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Sample records for mucopolysaccharidosis i

  1. Orthopedic Pathology in Children with Mucopolysaccharidosis Type I

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    Nato D. Vashakmadze

    2016-01-01

    Full Text Available Mucopolysaccharidosis type I is inherited in an autosomal recessive manner and results from the defective activity of the enzyme alpha-L-iduronidase, which leads to the accumulation of glycosaminoglycans (mainly heparan and dermatan sulfate in the lysosomes and further multiple organ dysfunction. This severe genetic progressive disease can be detected at an early age by skeletal deformities and phenotypic data. Early enzyme replacement therapy and/or bone marrow transplantation can slow down irreversible damages to various organs and systems or reduce their severity and improve the quality of life for a child.

  2. Mucopolysaccharidosis type I: current knowledge on its pathophysiological mechanisms.

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    Campos, Derbis; Monaga, Madelyn

    2012-06-01

    Mucopolysaccharidosis type I is one of the most frequent lysosomal storage diseases. It has a high morbidity and mortality, causing in many cases severe neurological and somatic damage in the first years of life. Although the clinical phenotypes have been described for decades, and the enzymatic deficiency and many of the mutations that cause this disease are well known, the underlying pathophysiological mechanisms that lead to its development are not completely understood. In this review we describe and discuss the different pathogenic mechanisms currently proposed for this disease regarding its neurological damage. Deficiency in the lysosomal degradation of heparan sulfate and dermatan sulfate, as well as its primary accumulation, may disrupt a variety of physiological and biochemical processes: the intracellular and extracellular homeostasis of these macromolecules, the pathways related to gangliosides metabolism, mechanisms related to the activation of inflammation, receptor-mediated signaling, oxidative stress and permeability of the lysosomal membrane, as well as alterations in intracellular ionic homeostasis and the endosomal pathway. Many of the pathogenic mechanisms proposed for mucopolysaccharidosis type I are also present in other lysosomal storage diseases with neurological implications. Results from the use of methods that allow the analysis of multiple genes and proteins, in both patients and animal models, will shed light on the role of each of these mechanisms and their combination in the development of different phenotypes due to the same deficiency.

  3. Genetics Home Reference: mucopolysaccharidosis type I

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    ... accumulation of GAGs within cells, specifically inside the lysosomes . Lysosomes are compartments in the cell that digest and ... that cause molecules to build up inside the lysosomes, including MPS I, are called lysosomal storage disorders. ...

  4. Phenotype prediction for mucopolysaccharidosis type I by in silico analysis.

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    Ou, Li; Przybilla, Michael J; Whitley, Chester B

    2017-07-04

    Mucopolysaccharidosis type I (MPS I) is an autosomal recessive disease due to deficiency of α-L-iduronidase (IDUA), a lysosomal enzyme that degrades glycosaminoglycans (GAG) heparan and dermatan sulfate. To achieve optimal clinical outcomes, early and proper treatment is essential, which requires early diagnosis and phenotype severity prediction. To establish a genotype/phenotype correlation of MPS I disease, a combination of bioinformatics tools including SIFT, PolyPhen, I-Mutant, PROVEAN, PANTHER, SNPs&GO and PHD-SNP are utilized. Through analyzing single nucleotide polymorphisms (SNPs) by these in silico approaches, 28 out of 285 missense SNPs were predicted to be damaging. By integrating outcomes from these in silico approaches, a prediction algorithm (sensitivity 94%, specificity 80%) was thereby developed. Three dimensional structural analysis of 5 candidate SNPs (P533R, P496R, L346R, D349G, T374P) were performed by SWISS PDB viewer, which revealed specific structural changes responsible for the functional impacts of these SNPs. Additionally, SNPs in the untranslated region were analyzed by UTRscan and PolymiRTS. Moreover, by investigating known pathogenic mutations and relevant patient phenotypes in previous publications, phenotype severity (severe, intermediate or mild) of each mutation was deduced. Collectively, these results identified potential candidate SNPs with functional significance for studying MPS I disease. This study also demonstrates the effectiveness, reliability and simplicity of these in silico approaches in addressing complexity of underlying genetic basis of MPS I disease. Further, a step-by-step guideline for phenotype prediction of MPS I disease is established, which can be broadly applied in other lysosomal diseases or genetic disorders.

  5. Heparan sulfate inhibits hematopoietic stem and progenitor cell migration and engraftment in mucopolysaccharidosis I.

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    Watson, H.A.; Holley, R.J.; Langford-Smith, K.J.; Wilkinson, F.L.; Kuppevelt, T.H. van; Wynn, R.F.; Wraith, J.E.; Merry, C.L.; Bigger, B.W.

    2014-01-01

    Mucopolysaccharidosis I Hurler (MPSI-H) is a pediatric lysosomal storage disease caused by genetic deficiencies in IDUA, coding for alpha-l-iduronidase. Idua(-/-) mice share similar clinical pathology with patients, including the accumulation of the undegraded glycosaminoglycans (GAGs) heparan

  6. A novel, long-lived, and highly engraftable immunodeficient mouse model of mucopolysaccharidosis type I

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    Daniel C Mendez

    Full Text Available Mucopolysaccharidosis type I (MPS I is an inherited α-L-iduronidase (IDUA, I deficiency in which glycosaminoglycan (GAG accumulation causes progressive multisystem organ dysfunction, neurological impairment, and death. Current MPS I mouse models, based on a NOD/SCID (NS background, are short-lived, providing a very narrow window to assess the long-term efficacy of therapeutic interventions. They also develop thymic lymphomas, making the assessment of potential tumorigenicity of human stem cell transplantation problematic. We therefore developed a new MPS I model based on a NOD/SCID/Il2rγ (NSG background. This model lives longer than 1 year and is tumor-free during that time. NSG MPS I (NSGI mice exhibit the typical phenotypic features of MPS I including coarsened fur and facial features, reduced/abnormal gait, kyphosis, and corneal clouding. IDUA is undetectable in all tissues examined while GAG levels are dramatically higher in most tissues. NSGI brain shows a significant inflammatory response and prominent gliosis. Neurological MPS I manifestations are evidenced by impaired performance in behavioral tests. Human neural and hematopoietic stem cells were found to readily engraft, with human cells detectable for at least 1 year posttransplantation. This new MPS I model is thus suitable for preclinical testing of novel pluripotent stem cell-based therapy approaches.

  7. Features of Brain MRI in Dogs with Treated and Untreated Mucopolysaccharidosis Type I

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    Vite, Charles H; Nestrasil, Igor; Mlikotic, Anton; Jens, Jackie K; Snella, Elizabeth M; Gross, William; Shapiro, Elsa G; Kovac, Victor; Provenzale, James M; Chen, Steven; Le, Steven Q; Kan, Shih-hsin; Banakar, Shida; Wang, Raymond Y; Haskins, Mark E; Ellinwood, N Matthew; Dickson, Patricia I

    2013-01-01

    The mucopolysaccharidosis type I (MPS I) dog model has been important in the development of therapies for human patients. We treated dogs with enzyme replacement therapy (ERT) by various approaches. Dogs assessed included untreated MPS I dogs, heterozygous carrier dogs, and MPS I dogs treated with intravenous ERT as adults (beginning at age 13 to 16 mo), intrathecal and intravenous ERT as adults (beginning at age 13 to 16 mo), or intrathecal ERT as juveniles (beginning at age 4 mo). We then characterized the neuroimaging findings of 32 of these dogs (age, 12 to 30 mo). Whole and midsagittal volumes of the corpus callosum, measured from brain MRI, were significantly smaller in affected dogs compared with unaffected heterozygotes. Corpus callosum volumes in dogs that were treated with intrathecal ERT from 4 mo until 21 mo of age were indistinguishable from those of age-matched carrier controls. Dogs with MPS I showed cerebral ventricular enlargement and cortical atrophy as early as 12 mo of age. Ventricular enlargement was greater in untreated MPS I dogs than in age-matched dogs treated with intrathecal ERT as juveniles or adults. However, treated dogs still showed some ventricular enlargement or cortical atrophy (or both). Understanding the progression of neuroimaging findings in dogs with MPS I and their response to brain-directed therapy may improve preclinical studies for new human-directed therapies. In particular, corpus callosum volumes may be useful quantitative neuroimaging markers for MPS-related brain disease and its response to therapy. PMID:23582423

  8. Impaired Hematopoiesis and Disrupted Monocyte/Macrophage Homeostasis in Mucopolysaccharidosis Type I Mice.

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    Viana, Gustavo Monteiro; Buri, Marcus Vinícius; Paredes-Gamero, Edgar Julian; Martins, Ana Maria; D'Almeida, Vânia

    2016-03-01

    Mucopolysaccharidosis type I (MPS I) is a rare autosomal recessive disease caused by alpha-L-iduronidase deficiency in which heparan and dermatan sulfate degradation is compromised. Besides primary lysosomal glycosaminoglycan accumulation, further changes in cellular functions have also been described in several murine MPS models. Herein, we evaluated alterations in hematopoiesis and its implications on the production of mature progeny in a MPS I murine model. Despite the significant increase in hematopoietic stem cells, a reduction in common myeloid progenitors and granulocyte-macrophage progenitor cells was observed in Idua -/- mice bone marrow. Furthermore, no alterations in number, viability nor activation of cell death mechanisms were observed in Idua -/- mice mature macrophages but they presented higher sensitivity to apoptotic induction after staurosporine treatment. In addition, changes in Ca(2+) signaling and a reduction in phagocytosis ability were also found. In summary, our results revealed significant intracellular changes in mature Idua -/- macrophages related to alterations in Idua -/- mice hematopoiesis, revealing a disruption in cell homeostasis. These results provide new insights into physiopathology of MPS I. © 2015 Wiley Periodicals, Inc.

  9. Neonatal bone marrow transplantation prevents bone pathology in a mouse model of mucopolysaccharidosis type I.

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    Pievani, Alice; Azario, Isabella; Antolini, Laura; Shimada, Tsutomu; Patel, Pravin; Remoli, Cristina; Rambaldi, Benedetta; Valsecchi, Maria Grazia; Riminucci, Mara; Biondi, Andrea; Tomatsu, Shunji; Serafini, Marta

    2015-03-05

    Neonatal bone marrow transplantation (BMT) could offer a novel therapeutic opportunity for genetic disorders by providing sustainable levels of the missing protein at birth, thus preventing tissue damage. We tested this concept in mucopolysaccharidosis type I (MPS IH; Hurler syndrome), a lysosomal storage disorder caused by deficiency of α-l-iduronidase. MPS IH is characterized by a broad spectrum of clinical manifestations, including severe progressive skeletal abnormalities. Although BMT increases the life span of patients with MPS IH, musculoskeletal manifestations are only minimally responsive if the timing of BMT delays, suggesting already irreversible bone damage. In this study, we tested the hypothesis that transplanting normal BM into newborn MPS I mice soon after birth can prevent skeletal dysplasia. We observed that neonatal BMT was effective at restoring α-l-iduronidase activity and clearing elevated glycosaminoglycans in blood and multiple organs. At 37 weeks of age, we observed an almost complete normalization of all bone tissue parameters, using radiographic, microcomputed tomography, biochemical, and histological analyses. Overall, the magnitude of improvements correlated with the extent of hematopoietic engraftment. We conclude that BMT at a very early stage in life markedly reduces signs and symptoms of MPS I before they appear. © 2015 by The American Society of Hematology.

  10. Altered corneal stromal matrix organization is associated with mucopolysaccharidosis I, III and VI.

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    Alroy, J; Haskins, M; Birk, D E

    1999-05-01

    The presence of cloudy corneas is a prominent feature of mucopolysaccharidosis (MPS) types I and VI, but not MPS IIIA or IIIB. The cause of corneal cloudiness in MPS I and VI is speculative. Transparency of the cornea is dependent on the uniform diameter and the regular spacing and arrangement of the collagen fibrils within the stroma. Alterations in the spacing of collagen fibrils in a variety of conditions including corneal edema, scars, and macular corneal dystrophy is clinically manifested as corneal opacity. The purpose of this study was to compare the structural organization of the stromal extracellular matrix of normal corneas with that of MPS corneas. The size and arrangement of collagen fibrils in cloudy corneas from patients with MPS I were examined. The alterations observed were an increased mean fibril diameter with a broader distribution in the MPS corneas. The MPS I corneas also had altered fibril spacing and more irregular packing compared with normal control corneas. The clear corneas of patients with MPS IIIA and IIIB also showed increases in mean fibril diameter and fibril spacing. However, there was less variation indicating more regularity than seen in MPS I. In addition, corneas from cat models of certain MPS were compared to the human corneas. Cats with MPS I and VI, as well as normal control cats, were examined. Structural alterations comparable to those seen in human MPS corneas were seen in MPS I and VI cats relative to normal clear corneas. The findings suggest that cloudy corneas in MPS I and VI are in part a consequence of structural alterations in the corneal stroma, including abnormal spacing, size, and arrangement of collagen fibrils. Copyright 1999 Academic Press.

  11. Mucopolysaccharidosis type I: molecular characteristics of two novel alpha-L-iduronidase mutations in Tunisian patients

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    Chahed Henda

    2011-06-01

    Full Text Available Abstract Background Mucopolysaccharidosis type I (MPS I is an autosomal storage disease resulting from defective activity of the enzyme α-L-iduronidase (IDUA. This glycosidase is involved in the degradation of heparan sulfate and dermatan sulfate. MPS I has severe and milder phenotypic subtypes. Aim of study: This study was carried out on six newly collected MPS I patients recruited from many regions of Tunisia. Patients and methods: Mutational analysis of the IDUA gene in unrelated MPS I families was performed by sequencing the exons and intron-exon junctions of IDUA gene. Results Two novel IDUA mutations, p.L530fs (1587_1588 insGC in exon 11 and p.F177S in exon 5 and two previously reported mutations p.P533R and p.Y581X were detected. The patient in family 1 who has the Hurler phenotype was homozygous for the previously described nonsense mutation p.Y581X. The patient in family 2 who also has the Hurler phenotype was homozygous for the novel missense mutation p.F177S. The three patients in families 3, 5 and 6 were homozygous for the p.P533R mutation. The patient in family 4 was homozygous for the novel small insertion 1587_1588 insGC. In addition, eighteen known and one unknown IDUA polymorphisms were identified. Conclusion The identification of these mutations should facilitate prenatal diagnosis and counseling for MPS I in Tunisia. Background Mucopolysaccharidosis type I (MPS I is an autosomal recessive lysosomal storage disorder caused by the deficient activity of the enzyme of α-L-iduronidase (IDUA, EC 3.2.1.76. This glycosidase is involved in the degradation of heparan sulfate and dermatan sulfate. The clinical phenotype of MPS I ranges from the very severe in Hurler syndrome (MPS IH to the relatively benign in Scheie syndrome (MPS IS, with an intermediate phenotype designated Hurler/Scheie (MPS IH/S 1. Isolation of complementary and genomic DNAs encoding human α -L- iduronidase 23 have enable the identification of mutations underlying

  12. Enzyme replacement therapy for Mucopolysaccharidosis Type I among patients followed within the MPS Brazil Network

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    Alícia Dorneles Dornelles

    2014-01-01

    Full Text Available Mucopolysaccharidosis type I (MPS I is a rare lysosomal disorder caused by deficiency of alph-L-iduronidase. Few clinical trials have assessed the effect of enzyme replacement therapy (ERT for this condition. We conducted an exploratory, open-label, non-randomized, multicenter cohort study of patients with MPS I. Data were collected from questionnaires completed by attending physicians at the time of diagnosis (T1; n = 34 and at a median time of 2.5 years later (T2; n = 24/34. The 24 patients for whom data were available at T2 were allocated into groups: A, no ERT (9 patients; median age at T1 = 36 months; 6 with severe phenotype; B, on ERT (15 patients; median age at T1 = 33 months; 4 with severe phenotype. For all variables in which there was no between-group difference at baseline, a delta of ;±20% was considered clinically relevant. The following clinically relevant differences were identified in group B in T2: lower rates of mortality and reported hospitalization for respiratory infection; lower frequency of hepatosplenomegaly; increased reported rates of obstructive sleep apnea syndrome and hearing loss; and stabilization of gibbus deformity. These changes could be due to the effect of ERT or of other therapies which have also been found more frequently in group B. Our findings suggest MPS I patients on ERT also receive a better overall care. ERT may have a positive effect on respiratory morbidity and overall mortality in patients with MPS I. Additional studies focusing on these outcomes and on other therapies should be performed.

  13. Does early use of enzyme replacement therapy alter the natural history of mucopolysaccharidosis I? Experience in three siblings.

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    Laraway, Sarah; Breen, Catherine; Mercer, Jean; Jones, Simon; Wraith, James E

    2013-07-01

    Enzyme replacement therapy is widely used as treatment for mucopolysaccharidosis I (MPS I), and there is evidence that this produces improvement in certain clinical domains. There does appear to be variation in the response of clinical features to treatment once these are established. In a reported sibling pair, when enzyme replacement therapy was commenced pre-symptomatically in the younger child, the natural history of the condition appeared to be affected. We present data from three siblings treated with enzyme replacement therapy at different ages which supports this finding. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. Treatment of hip dysplasia in patients with mucopolysaccharidosis type I after hematopoietic stem cell transplantation: results of an international consensus procedure

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    Langereis, Eveline J.; Borgo, Andrea; Crushell, Ellen; Harmatz, Paul R.; van Hasselt, Peter M.; Jones, Simon A.; Kelly, Paula M.; Lampe, Christina; van der Lee, Johanna H.; Odent, Thierry; Sakkers, Ralph; Scarpa, Maurizio; Schafroth, Matthias U.; Struijs, Peter A.; Valayannopoulos, Vassili; White, Klane K.; Wijburg, Frits A.

    2013-01-01

    Mucopolysaccharidosis type I (MPS-I) is a lysosomal storage disorder characterized by progressive multi-organ disease. The standard of care for patients with the severe phenotype (Hurler syndrome, MPS I-H) is early hematopoietic stem cell transplantation (HSCT). However, skeletal disease, including

  15. Extracellular matrix disruption is an early event in the pathogenesis of skeletal disease in mucopolysaccharidosis I.

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    Heppner, Jonathan M; Zaucke, Frank; Clarke, Lorne A

    2015-02-01

    Progressive skeletal and connective tissue disease represents a significant clinical burden in all of the mucopolysaccharidoses. Despite the introduction of enzyme replacement strategies for many of the mucopolysaccharidoses, symptomatology related to bone and joint disease appears to be recalcitrant to current therapies. In order to address these unmet medical needs a clearer understanding of skeletal and connective tissue disease pathogenesis is required. Historically the pathogenesis of the mucopolysaccharidoses has been assumed to directly relate to progressive storage of glycosaminoglycans. It is now apparent for many lysosomal storage disorders that more complex pathogenic mechanisms underlie patients' clinical symptoms. We have used proteomic and genome wide expression studies in the murine mucopolysaccharidosis I model to identify early pathogenic events occurring in micro-dissected growth plate tissue. Studies were conducted using 3 and 5-week-old mice thus representing a time at which no obvious morphological changes of bone or joints have taken place. An unbiased iTRAQ differential proteomic approach was used to identify candidates followed by validation with multiple reaction monitoring mass spectrometry and immunohistochemistry. These studies reveal significant decreases in six key structural and signaling extracellular matrix proteins; biglycan, fibromodulin, PRELP, type I collagen, lactotransferrin, and SERPINF1. Genome-wide expression studies in embryonic day 13.5 limb cartilage and 5 week growth plate cartilage followed by specific gene candidate qPCR studies in the 5week growth plate identified fourteen significantly deregulated mRNAs (Adamts12, Aspn, Chad, Col2a1, Col9a1, Hapln4, Lum, Matn1, Mmp3, Ogn, Omd, P4ha2, Prelp, and Rab32). The involvement of biglycan, PRELP and fibromodulin; all members of the small leucine repeat proteoglycan family is intriguing, as this protein family is implicated in the pathogenesis of late onset osteoarthritis

  16. The diagnostic journey of patients with mucopolysaccharidosis I: A real-world survey of patient and physician experiences

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    Stefano Bruni

    2016-09-01

    Full Text Available Mucopolysaccharidosis type I (MPS I is an inherited lysosomal storage disease. Affected individuals have disease ranging from attenuated to severe with significant disease burden, disability, and premature death. Early treatment with enzyme replacement therapy and/or stem cell transplantation can reduce disease progression and improve outcomes. However, diagnosis is often delayed, particularly for patients with attenuated phenotypes. We conducted a survey of 168 patients and 582 physicians to explore health care seeking patterns and familiarity of physicians with MPS I symptoms. Patients with attenuated MPS I typically first presented with stiff joints or hernia/bulging abdomen, and patients with severe disease with noisy/difficult breathing, or hernia/bulging abdomen. There was a mean delay from time of symptom presentation to diagnosis of 2.7 years for patients with attenuated disease, with a mean of 5 physicians consulted before receiving a correct diagnosis. MPS I was most commonly misidentified by physicians as rheumatoid arthritis (48–72%, with a wide variety of suspected diseases, including lupus. CONCLUSION: Patient and physician real-world surveys show that MPS I is under-recognized and diagnosis of MPS I remains delayed, particularly in patients with attenuated disease. Across regions and specialties, physicians require differential diagnosis education in order to improve early detection and early treatment initiation of MPS I.

  17. Mucopolysaccharidosis VI

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    Harmatz Paul

    2010-04-01

    Full Text Available Abstract Mucopolysaccharidosis VI (MPS VI is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B leading to the accumulation of dermatan sulfate. Birth prevalence is between 1 in 43,261 and 1 in 1,505,160 live births. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms. The characteristic skeletal dysplasia includes short stature, dysostosis multiplex and degenerative joint disease. Rapidly progressing forms may have onset from birth, elevated urinary glycosaminoglycans (generally >100 μg/mg creatinine, severe dysostosis multiplex, short stature, and death before the 2nd or 3rd decades. A more slowly progressing form has been described as having later onset, mildly elevated glycosaminoglycans (generally ARSB gene, located in chromosome 5 (5q13-5q14. Over 130 ARSB mutations have been reported, causing absent or reduced arylsulfatase B (N-acetylgalactosamine 4-sulfatase activity and interrupted dermatan sulfate and chondroitin sulfate degradation. Diagnosis generally requires evidence of clinical phenotype, arylsulfatase B enzyme activity ®, clinical management was limited to supportive care and hematopoietic stem cell transplantation. Galsulfase is now widely available and is a specific therapy providing improved endurance with an acceptable safety profile. Prognosis is variable depending on the age of onset, rate of disease progression, age at initiation of ERT and on the quality of the medical care provided.

  18. Intraperitoneal implant of recombinant encapsulated cells overexpressing alpha-L-iduronidase partially corrects visceral pathology in mucopolysaccharidosis type I mice.

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    Baldo, Guilherme; Mayer, Fabiana Quoos; Martinelli, Barbara; Meyer, Fabiola Schons; Burin, Maira; Meurer, Luise; Tavares, Angela Maria Vicente; Giugliani, Roberto; Matte, Ursula

    2012-08-01

    Mucopolysaccharidosis type I (MPS I) is characterized by deficiency of the enzyme alpha-L-iduronidase (IDUA) and storage of glycosaminoglycans (GAG) in several tissues. Current available treatments present limitations, thus the search for new therapies. Encapsulation of recombinant cells within polymeric structures combines gene and cell therapy and is a promising approach for treating MPS I. We produced alginate microcapsules containing baby hamster kidney (BHK) cells overexpressing IDUA and implanted these capsules in the peritoneum of MPS I mice. An increase in serum and tissue IDUA activity was observed at early time-points, as well as a reduction in GAG storage; however, correction in the long term was only partially achieved, with a drop in the IDUA activity being observed a few weeks after the implant. Analysis of the capsules obtained from the peritoneum revealed inflammation and a pericapsular fibrotic process, which could be responsible for the reduction in IDUA levels observed in the long term. In addition, treated mice developed antibodies against the enzyme. The results suggest that the encapsulation process is effective in the short term but improvements must be achieved in order to reduce the immune response and reach a stable correction.

  19. Factors influencing transfection efficiency of pIDUA/nanoemulsion complexes in a mucopolysaccharidosis type I murine model

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    Fraga M

    2017-03-01

    Full Text Available Michelle Fraga,1,2 Talita Giacomet de Carvalho,2,3 Juliana Bidone,1 Roselena Silvestri Schuh,1,2 Ursula Matte,2,3 Helder Ferreira Teixeira1 1Pharmaceutical Sciences Graduate Program, Universidade Federal do Rio Grande do Sul, 2Gene Therapy Center, Experimental Research Center, Hospital de Clínicas de Porto Alegre, 3Genetics and Molecular Biology Graduate Program, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil Abstract: Mucopolysaccharidosis type I (MPS I is an autosomal disease caused by alpha-L-iduronidase (IDUA deficiency. This study used IDUA knockout mice as a model to evaluate whether parameters such as dose of plasmid and time of treatment could influence the transfection efficiency of complexes formed with PEGylated cationic nanoemulsions and plasmid (pIDUA, which contains the gene that encodes for IDUA. Formulations were composed of medium chain triglycerides, 1,2-dioleoyl-sn-glycero-3-phosphoethanolamine, 1,2-distearoyl-sn-glycero-3-phosphoethanolamine-N-(amino[polyethylene glycol]-2000, 1,2-dioleoyl-sn-glycero-3-trimethylammonium propane (DOTAP, glycerol, and water and were prepared by the adsorption or encapsulation of preformed pIDUA–DOTAP complexes by high-pressure homogenization. A progressive increase in IDUA expression was observed with an increase in the dose and time of transfection for mice treated with both complexes (adsorbed and encapsulated, especially in the liver. Regardless of the complex administered, a significant increase in IDUA activity was detected in lungs and liver compared with nontreated MPS I when a dose of 60 µg was administered and IDUA activity was measured 7 days postadministration. Tissue sections of major organs showed no presence of cell necrosis, inflammatory infiltrate, or an increase in apoptosis. Furthermore, immunohistochemistry for CD68 showed no difference in the number of macrophage cells in treated and nontreated animals, indicating the absence of inflammatory reaction

  20. Diagnosing mucopolysaccharidosis IVA

    NARCIS (Netherlands)

    T.C. Wood (Timothy); K. Harvey (Kirsten); M. Beck (Markus); M.G. Burin (Maira Graeff); Y.H. Chien; H.J. Church (Heather); V. D'Almeida (Vânia); O.P. van Diggelen (Otto); M. Fietz (Michael); R. Giugliani (Roberto); P. Harmatz (Paul); S.M. Hawley (Sara); W.L. Hwu; D. Ketteridge (David); Z. Lukacs; N. Miller (Nicola); M. Pasquali (Marzia); A. Schenone (Andrea); J.N. Thompson; K. Tylee (Karen); C. Yu (Cong); C. Hendriksz (Chris)

    2013-01-01

    textabstractMucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is an autosomal recessive lysosomal storage disorder resulting from a deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS) activity. Diagnosis can be challenging and requires agreement of clinical, radiographic, and

  1. Elevated TNF-α is associated with pain and physical disability in mucopolysaccharidosis types I, II, and VI.

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    Polgreen, Lynda E; Vehe, Richard K; Rudser, Kyle; Kunin-Batson, Alicia; Utz, Jeanine Jarnes; Dickson, Patricia; Shapiro, Elsa; Whitley, Chester B

    2016-04-01

    Children and adults with the lysosomal storage diseases mucopolysaccharidosis (MPS) types I, II and VI live shortened lives permeated by chronic pain and physical disability. Current treatments do not alleviate these problems. Thus there is a critical need to understand the mechanism of chronic pain and disability in MPS in order to improve the way we treat patients. A potential target is inflammation. We hypothesized that excessive inflammation mediated by the tumor necrosis factor-α (TNF-α) inflammatory pathway is the fundamental cause of much of the chronic pain and physical disability in MPS. 55 patients with MPS I, II, or VI were enrolled over the course of a 5-year prospective longitudinal natural history study and evaluated annually for 2-5years. 51 healthy controls were enrolled in a separate cross-sectional study of bone and energy metabolism. TNF-α was measured by ELISA. Pain and physical disability were measured by the Children's Health Questionnaire - Parent Form 50 (CHQ-PF50). Differences in log-transformed TNF-α levels and associations with CHQ domains were evaluated using a linear mixed effects model with random intercept. TNF-α levels were measured in 48 MPS (age: 5-17years; 35% female) and 51 controls (age: 8-17years; 53% female). Among MPS, 22 (46%) were treated with hematopoietic cell transplantation (HCT) alone, 24 (50%) with enzyme replacement therapy (ERT) alone, and 2 (4%) with both HCT and ERT. TNF-α levels are higher in MPS compared to healthy controls (p<0.001). Higher TNF-α levels are associated with increased pain and decreased physical function, social limitations due to physical health, and physical summary score (all p<0.05). TNF-α levels were not significantly associated with the general health score. TNF-α levels did not change significantly over time in MPS. Higher TNF-α levels are implicated in the pain and decreased physical function present in individuals with MPS despite treatment with ERT and/or HCT, suggesting that

  2. Mucopolysaccharidosis I, II, and VI: Brief review and guidelines for treatment.

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    Giugliani, Roberto; Federhen, Andressa; Rojas, Maria Verônica Muñoz; Vieira, Taiane; Artigalás, Osvaldo; Pinto, Louise Lapagesse; Azevedo, Ana Cecília; Acosta, Angelina; Bonfim, Carmen; Lourenço, Charles Marques; Kim, Chong Ae; Horovitz, Dafne; Bonfim, Denize; Norato, Denise; Marinho, Diane; Palhares, Durval; Santos, Emerson Santana; Ribeiro, Erlane; Valadares, Eugênia; Guarany, Fábio; de Lucca, Gisele Rosone; Pimentel, Helena; de Souza, Isabel Neves; Correa, Jordão; Fraga, José Carlos; Goes, José Eduardo; Cabral, José Maria; Simionato, José; Llerena, Juan; Jardim, Laura; Giuliani, Liane; da Silva, Luiz Carlos Santana; Santos, Mara L; Moreira, Maria Angela; Kerstenetzky, Marcelo; Ribeiro, Márcia; Ruas, Nicole; Barrios, Patricia; Aranda, Paulo; Honjo, Rachel; Boy, Raquel; Costa, Ronaldo; Souza, Carolina; Alcantara, Flavio F; Avilla, Silvio Gilberto A; Fagondes, Simone; Martins, Ana Maria

    2010-10-01

    Mucopolysaccharidoses (MPS) are rare genetic diseases caused by the deficiency of one of the lysosomal enzymes involved in the glycosaminoglycan (GAG) breakdown pathway. This metabolic block leads to the accumulation of GAG in various organs and tissues of the affected patients, resulting in a multisystemic clinical picture, sometimes including cognitive impairment. Until the beginning of the XXI century, treatment was mainly supportive. Bone marrow transplantation improved the natural course of the disease in some types of MPS, but the morbidity and mortality restricted its use to selected cases. The identification of the genes involved, the new molecular biology tools and the availability of animal models made it possible to develop specific enzyme replacement therapies (ERT) for these diseases. At present, a great number of Brazilian medical centers from all regions of the country have experience with ERT for MPS I, II, and VI, acquired not only through patient treatment but also in clinical trials. Taking the three types of MPS together, over 200 patients have been treated with ERT in our country. This document summarizes the experience of the professionals involved, along with the data available in the international literature, bringing together and harmonizing the information available on the management of these severe and progressive diseases, thus disclosing new prospects for Brazilian patients affected by these conditions.

  3. Mucopolysaccharidosis I, II, and VI: brief review and guidelines for treatment

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    Roberto Giugliani

    2010-01-01

    Full Text Available Mucopolysaccharidoses (MPS are rare genetic diseases caused by the deficiency of one of the lysosomal enzymes involved in the glycosaminoglycan (GAG breakdown pathway. This metabolic block leads to the accumulation of GAG in various organs and tissues of the affected patients, resulting in a multisystemic clinical picture, sometimes including cognitive impairment. Until the beginning of the XXI century, treatment was mainly supportive. Bone marrow transplantation improved the natural course of the disease in some types of MPS, but the morbidity and mortality restricted its use to selected cases. The identification of the genes involved, the new molecular biology tools and the availability of animal models made it possible to develop specific enzyme replacement therapies (ERT for these diseases. At present, a great number of Brazilian medical centers from all regions of the country have experience with ERT for MPS I, II, and VI, acquired not only through patient treatment but also in clinical trials. Taking the three types of MPS together, over 200 patients have been treated with ERT in our country. This document summarizes the experience of the professionals involved, along with the data available in the international literature, bringing together and harmonizing the information available on the management of these severe and progressive diseases, thus disclosing new prospects for Brazilian patients affected by these conditions.

  4. Pilot study of the safety and effect of adalimumab on pain, physical function, and musculoskeletal disease in mucopolysaccharidosis types I and II

    Directory of Open Access Journals (Sweden)

    Lynda E. Polgreen

    2017-03-01

    Full Text Available Mucopolysaccharidosis I and II are lysosomal storage disorders that, despite treatment with hematopoietic cell transplantation (HCT and/or enzyme replacement therapy (ERT, continue to cause significant skeletal abnormalities leading to pain, stiffness, physical dysfunction, and short stature. Tumor necrosis factor – alpha (TNF-α is elevated in individuals with MPS I and II and associated with pain and physical dysfunction. Therefore, we evaluated the safety and effects of the TNF-α inhibitor adalimumab in patients with MPS I and II in a 32-week, randomized, double blind, placebo-controlled, crossover study of adalimumab at a dose of 20 mg (weight 15–5° in seven of eight joints in Subject #1 and five of eight joints in Subject #2 (range 7.0° to 52.8°. There was no change in the PPQ, 6MWT, or hand dynamometer. Data from this small pilot study suggest that treatment with adalimumab is safe, tolerable, and may improve ROM, physical function, and possibly pain, in children with MPS I or II. However, additional clinical trials are needed before this therapy should be recommended as part of clinical care.

  5. Metabolic Syndrome and Cardiovascular Risk Factors after Hematopoietic Cell Transplantation in Severe Mucopolysaccharidosis Type I (Hurler Syndrome).

    Science.gov (United States)

    Braunlin, Elizabeth; Steinberger, Julia; DeFor, Todd; Orchard, Paul; Kelly, Aaron S

    2018-02-01

    Hematopoietic cell transplantation is a life-saving procedure, but one associated with increasing long-term cardiovascular risk requiring frequent long-term follow-up. This therapy has significantly lengthened survival in mucopolysaccharidosis type IH (Hurler syndrome), a disease with known coronary artery involvement. Metabolic syndrome-a constellation of central obesity, high blood pressure, low high-density lipoprotein cholesterol, elevated triglycerides, and fasting blood glucose-is associated with increased cardiovascular risk, and occurs when any 3 or more of these 5 components is present within a single individual. The incidence of metabolic syndrome and its components is poorly defined after transplantation for Hurler syndrome. Chart review of all long-term survivors of hematopoietic cell transplantation for Hurler syndrome ≥9 years of age for factors comprising the metabolic syndrome: obesity, high blood pressure, low high-density lipoprotein cholesterol, elevated triglycerides, and fasting blood glucose. Sixty-three patients were evaluated, 20 of whom had components of the metabolic syndrome available for review. There was no significant difference in age at transplantation, sex, number of transplants, pretransplant radiation, or percent engraftment between those with and without these data. Median follow-up after transplantation for the 20 patients with data was 14.3 years. Only 1 (5%) patient of this group fulfilled the criteria for metabolic syndrome. Fifty-three percent of the patients had 1 or more components of metabolic syndrome: the most common was high blood pressure occurring in 40%. Metabolic syndrome is uncommon in this cohort of long-term survivors of hematopoietic cell transplantation for Hurler syndrome but almost half of the patients had 1 or more components of the syndrome, with high blood pressure being the most common. Further studies are needed to develop guidelines in this diagnosis as well as other nonmalignant diseases of children

  6. Genotypic and bioinformatic evaluation of the alpha-l-iduronidase gene and protein in patients with mucopolysaccharidosis type I from Colombia, Ecuador and Peru.

    Science.gov (United States)

    Pineda, Tatiana; Marie, Sulie; Gonzalez, Janneth; García, Ana L; Acosta, Amparo; Morales, Manuel; Correa, Luz N; Vivas, Ricardo; Escobar, Xiomara; Protzel, Ana; Barba, Maria; Ospina, Sandra; Corredor, Clara; Mansilla, Sandra; Velasco, Harvy M

    2014-01-01

    Mucopolysaccharidosis type I (MPSI) is a rare autosomal recessive disorder caused by mutations in the gene encoding the lysosomal enzyme α-l-iduronidase (IDUA), which is instrumental in the hydrolysis of the glycosaminoglycans, dermatan and heparan sulfate. The accumulation of unhydrolyzed glycosaminoglycans leads to pathogenesis in multiple tissue types, especially those of skeletal, nervous, respiratory, cardiovascular, and gastrointestinal origin. Although molecular diagnostic tools for MPSI have been available since the identification and characterization of the IDUA gene in 1992, Colombia, Ecuador, and Peru have lacked such methodologies. Therefore, the mutational profile of the IDUA gene in these countries has largely been unknown. The goal of this study was to characterize genotypes in 14 patients with MPSI from Colombia, Ecuador, and Peru. The most common mutation found at a frequency of 42.8% was W402X. Six patients presented with seven novel mutations, a high novel mutational rate in this population (32%). These novel mutations were validated using bioinformatic techniques. A model of the IDUA protein resulting from three of the novel missense mutations (Y625C, P385L, R621L) revealed that these mutations alter accessible surface area values, thereby reducing the accessibility of the enzyme to its substrates. This is the first characterization of the mutational profile of the IDUA gene in patients with MPSI in Colombia, Ecuador, and Peru. The findings contribute to our understanding of IDUA gene expression and IDUA enzyme function, and may help facilitate early and improved diagnosis and management for patients with MPSI.

  7. Low-cost, rapidly-developed, 3D printed in vitro corpus callosum model for mucopolysaccharidosis type I [version 2; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Anthony Tabet

    2017-03-01

    Full Text Available The rising prevalence of high throughput screening and the general inability of (1 two dimensional (2D cell culture and (2 in vitro release studies to predict in vivo neurobiological and pharmacokinetic responses in humans has led to greater interest in more realistic three dimensional (3D benchtop platforms. Advantages of 3D human cell culture over its 2D analogue, or even animal models, include taking the effects of microgeometry and long-range topological features into consideration. In the era of personalized medicine, it has become increasingly valuable to screen candidate molecules and synergistic therapeutics at a patient-specific level, in particular for diseases that manifest in highly variable ways. The lack of established standards and the relatively arbitrary choice of probing conditions has limited in vitro drug release to a largely qualitative assessment as opposed to a predictive, quantitative measure of pharmacokinetics and pharmacodynamics in tissue. Here we report the methods used in the rapid, low-cost development of a 3D model of a mucopolysaccharidosis type I patient’s corpus callosum, which may be used for cell culture and drug release. The CAD model is developed from in vivo brain MRI tracing of the corpus callosum using open-source software, printed with poly (lactic-acid on a Makerbot Replicator 5X, UV-sterilized, and coated with poly (lysine for cellular adhesion. Adaptations of material and 3D printer for expanded applications are also discussed.

  8. Enzyme Replacement Therapy and/or Hematopoietic Stem Cell Transplantation at diagnosis in patients with Mucopolysaccharidosis type I: results of a European consensus procedure

    LENUS (Irish Health Repository)

    de Ru, Minke H

    2011-08-10

    Abstract Background Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder that results in the accumulation of glycosaminoglycans causing progressive multi-organ dysfunction. Its clinical spectrum is very broad and varies from the severe Hurler phenotype (MPS I-H) which is characterized by early and progressive central nervous system (CNS) involvement to the attenuated Scheie phenotype (MPS I-S) with no CNS involvement. Indication, optimal timing, safety and efficacy of the two available treatment options for MPS I, enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT), are subject to continuing debate. A European consensus procedure was organized to reach consensus about the use of these two treatment strategies. Methods A panel of specialists, including 8 specialists for metabolic disorders and 7 bone marrow transplant physicians, all with acknowledged expertise in MPS I, participated in a modified Delphi process to develop consensus-based statements on MPS I treatment. Fifteen MPS I case histories were used to initiate the discussion and to anchor decisions around either treatment mode. Before and at the meeting all experts gave their opinion on the cases (YES\\/NO transplantation) and reasons for their decisions were collected. A set of draft statements on MPS I treatment options composed by a planning committee were discussed and revised during the meeting until full consensus. Results Full consensus was reached on several important issues, including the following: 1) The preferred treatment for patients with MPS I-H diagnosed before age 2.5 yrs is HSCT; 2) In individual patients with an intermediate phenotype HSCT may be considered if there is a suitable donor. However, there are no data on efficacy of HSCT in patients with this phenotype; 3) All MPS I patients including those who have not been transplanted or whose graft has failed may benefit significantly from ERT; 4) ERT should be started at diagnosis and may be

  9. Intra-articular enzyme replacement therapy with rhIDUA is safe, well-tolerated, and reduces articular GAG storage in the canine model of mucopolysaccharidosis type I.

    Science.gov (United States)

    Wang, Raymond Y; Aminian, Afshin; McEntee, Michael F; Kan, Shih-Hsin; Simonaro, Calogera M; Lamanna, William C; Lawrence, Roger; Ellinwood, N Matthew; Guerra, Catalina; Le, Steven Q; Dickson, Patricia I; Esko, Jeffrey D

    2014-08-01

    Treatment with intravenous enzyme replacement therapy and hematopoietic stem cell transplantation for mucopolysaccharidosis (MPS) type I does not address joint disease, resulting in persistent orthopedic complications and impaired quality of life. A proof-of-concept study was conducted to determine the safety, tolerability, and efficacy of intra-articular recombinant human iduronidase (IA-rhIDUA) enzyme replacement therapy in the canine MPS I model. Four MPS I dogs underwent monthly rhIDUA injections (0.58 mg/joint) into the right elbow and knee for 6 months. Contralateral elbows and knees concurrently received normal saline. No intravenous rhIDUA therapy was administered. Monthly blood counts, chemistries, anti-rhIDUA antibody titers, and synovial fluid cell counts were measured. Lysosomal storage of synoviocytes and chondrocytes, synovial macrophages and plasma cells were scored at baseline and 1 month following the final injection. All injections were well-tolerated without adverse reactions. One animal required prednisone for spinal cord compression. There were no clinically significant abnormalities in blood counts or chemistries. Circulating anti-rhIDUA antibody titers gradually increased in all dogs except the prednisone-treated dog; plasma cells, which were absent in all baseline synovial specimens, were predominantly found in synovium of rhIDUA-treated joints at study-end. Lysosomal storage in synoviocytes and chondrocytes following 6 months of IA-rhIDUA demonstrated significant reduction compared to tissues at baseline, and saline-treated tissues at study-end. Mean joint synovial GAG levels in IA-rhIDUA joints were 8.62 ± 5.86 μg/mg dry weight and 21.6 ± 10.4 μg/mg dry weight in control joints (60% reduction). Cartilage heparan sulfate was also reduced in the IA-rhIDUA joints (113 ± 39.5 ng/g wet weight) compared to saline-treated joints (142 ± 56.4 ng/g wet weight). Synovial macrophage infiltration, which was present in all joints at baseline, was

  10. Cerebral magnetic resonance findings during enzyme replacement therapy in mucopolysaccharidosis

    International Nuclear Information System (INIS)

    Matsubara, Yoshiko; Miyazaki, Osamu; Nosaka, Shunsuke; Kosuga, Motomichi; Okuyama, Torayuki

    2017-01-01

    Although enzyme replacement therapy (ERT) is an effective treatment for mucopolysaccharidosis (MPS) types I, II, IVA and VI, its effectiveness in children with central nervous system (CNS) disorders is said to be poor because the blood-brain barrier cannot be penetrated by ERT drugs. To assess CNS involvement in mucopolysaccharidosis at the start of enzyme replacement therapy and to investigate the time course of ERT in the central nervous system. We performed brain MRI in 17 children and young adults who underwent ERT. The clinical severity was classified as attenuated or severe by a specialist pediatrician, based on the clinical symptoms and genotypes. At the start of ERT, we scored nine parameters using two- or three-point scales based on the severity of the disease revealed on MRI scans. After the start of ERT, we compared the initial and follow-up MRI scans, and classified the findings as no change, improved or worse. We then compared the results with the changes in clinical findings. At the start of ERT, comparison of the clinical symptoms and image scores revealed differences between severe and attenuated mucopolysaccharidosis. The scores in patients with severe MPS ranged from 9 to 16 (mean 12.2); for patients with attenuated MPS, they ranged from 2 to 11 (mean 6.4). Images of the four patients with severe MPS showed ventricular dilation and brain atrophy. Such findings were made in only 2 of 13 patients with attenuated MPS. The results after the start of ERT showed that 11/17 (65%) patients manifested improvement or no change. All five patients with MPS I experienced improvement in some regions. There were no new lesions. One patient with MPS II experienced worsening of his CNS symptoms, and his MRI findings revealed more severe ventricular dilation, brain atrophy and white matter lesions. Ventricular dilation and brain atrophy on imaging studies might represent useful markers in predicting the severity of mucopolysaccharidosis and worsening of CNS

  11. Cerebral magnetic resonance findings during enzyme replacement therapy in mucopolysaccharidosis

    Energy Technology Data Exchange (ETDEWEB)

    Matsubara, Yoshiko [Division of Radiology, National Center for Child Health and Development, Tokyo (Japan); Hiroshima University, Department of Diagnostic Radiology, Hiroshima (Japan); Miyazaki, Osamu; Nosaka, Shunsuke [Division of Radiology, National Center for Child Health and Development, Tokyo (Japan); Kosuga, Motomichi [Division of Medical Genetics, National Center for Child Health and Development, Tokyo (Japan); Okuyama, Torayuki [Department of Clinical Laboratory Medicine, National Center for Child Health and Development, Tokyo (Japan)

    2017-11-15

    Although enzyme replacement therapy (ERT) is an effective treatment for mucopolysaccharidosis (MPS) types I, II, IVA and VI, its effectiveness in children with central nervous system (CNS) disorders is said to be poor because the blood-brain barrier cannot be penetrated by ERT drugs. To assess CNS involvement in mucopolysaccharidosis at the start of enzyme replacement therapy and to investigate the time course of ERT in the central nervous system. We performed brain MRI in 17 children and young adults who underwent ERT. The clinical severity was classified as attenuated or severe by a specialist pediatrician, based on the clinical symptoms and genotypes. At the start of ERT, we scored nine parameters using two- or three-point scales based on the severity of the disease revealed on MRI scans. After the start of ERT, we compared the initial and follow-up MRI scans, and classified the findings as no change, improved or worse. We then compared the results with the changes in clinical findings. At the start of ERT, comparison of the clinical symptoms and image scores revealed differences between severe and attenuated mucopolysaccharidosis. The scores in patients with severe MPS ranged from 9 to 16 (mean 12.2); for patients with attenuated MPS, they ranged from 2 to 11 (mean 6.4). Images of the four patients with severe MPS showed ventricular dilation and brain atrophy. Such findings were made in only 2 of 13 patients with attenuated MPS. The results after the start of ERT showed that 11/17 (65%) patients manifested improvement or no change. All five patients with MPS I experienced improvement in some regions. There were no new lesions. One patient with MPS II experienced worsening of his CNS symptoms, and his MRI findings revealed more severe ventricular dilation, brain atrophy and white matter lesions. Ventricular dilation and brain atrophy on imaging studies might represent useful markers in predicting the severity of mucopolysaccharidosis and worsening of CNS

  12. Prevalence of Mucopolysaccharidosis Types I, II, and VI in the Pediatric and Adult Population with Carpal Tunnel Syndrome (CTS). Retrospective and Prospective Analysis of Patients Treated for CTS

    DEFF Research Database (Denmark)

    Nørmark, Mette Borch; Kjaer, Nanna; Lund, Allan Meldgaard

    2017-01-01

    BACKGROUND: We wanted to investigate whether the prevalence of mucopolysaccharidoses (MPS) I, II, and VI was higher than expected in a selected cohort of patients with carpal tunnel syndrome (CTS). CTS is a common finding in patients with MPS, and therefore we screened patients who had undergone ...

  13. The impact of laronidase treatment in otolaryngological manifestations of patients with mucopolysaccharidosis.

    Science.gov (United States)

    Dualibi, Ana Paula Fiuza Funicello; Martins, Ana Maria; Moreira, Gustavo Antônio; de Azevedo, Marisa Frasson; Fujita, Reginaldo Raimundo; Pignatari, Shirley Shizue Nagata

    2016-01-01

    Mucopolysaccharidosis (MPS) is a lysosomal storage disease caused by deficiency of α-l-iduronidase. The otolaryngological findings include hearing loss, otorrhea, recurrent otitis, hypertrophy of tonsils and adenoid, recurrent rhinosinusitis, speech disorders, snoring, oral breathing and nasal obstruction. To evaluate the impact of enzymatic replacement therapy with laronidase (Aldurazyme(®)) in patients with mucopolysaccharidosis (MPS I), regarding sleep and hearing disorders, and clinical manifestations in the upper respiratory tract (URT). Nine patients with MPS I (8 Hurler-Scheie, and 1 Scheie phenotypes) of both sexes, ages ranging between 3 and 20 years, were included in this study. Patients were evaluated between seven and 11 months before the treatment and between 16 and 22 months after the onset of the enzymatic replacement. They were all submitted to a clinical and otolaryngological evaluation, including nasofibroscopical, polysomnographic and audiologic exams. The results' data showed decreasing of the frequency of ear, nose and throat infections, with improvement of the rhinorrhea and respiratory quality. No remarkable changes were observed regarding macroglossia and tonsil and adenoid hypertrophy. Audiometric and polysomnographic evaluations did not show statistical significance. Enzymatic replacement therapy in patients with mucopolysaccharidosis I provides control of recurrent URT infections, rhinorrhea and respiratory quality, however it is does not seem to improve audiologic and polisomnographic parameters, with no effect on adenoid and tonsils hypertrophy and macroglossia. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  14. Total Hip Arthroplasty in Mucopolysaccharidosis Type IH

    Directory of Open Access Journals (Sweden)

    S. O'hEireamhoin

    2011-01-01

    Full Text Available Children affected by mucopolysaccharidosis (MPS type IH (Hurler Syndrome, an autosomal recessive metabolic disorder, are known to experience a range of musculoskeletal manifestations including spinal abnormalities, hand abnormalities, generalised joint stiffness, genu valgum, and hip dysplasia and avascular necrosis. Enzyme therapy, in the form of bone marrow transplantation, significantly increases life expectancy but does not prevent the development of the associated musculoskeletal disorders. We present the case of a 23-year-old woman with a diagnosis of Hurler syndrome with a satisfactory result following uncemented total hip arthroplasty.

  15. Diagnostic and treatment strategies in mucopolysaccharidosis VI

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    Vairo F

    2015-10-01

    Full Text Available Filippo Vairo,1–3 Andressa Federhen,1,3,4 Guilherme Baldo,1,2,5–7 Mariluce Riegel,1,6 Maira Burin,1 Sandra Leistner-Segal,1,8 Roberto Giugliani1,5,6,81Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil; 2Department of Genetics, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil; 3Clinical Research Group on Medical Genetics, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil; 4Post-Graduate Program in Child and Adolescent Health, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil; 5Gene Therapy Center, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil; 6Post-Graduate Program in Genetics and Molecular Biology, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil; 7Department of Physiology, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil; 8Post-Graduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, BrazilAbstract: Mucopolysaccharidosis VI (MPS VI is a very rare autosomal recessive disorder caused by mutations in the ARSB gene, which lead to deficient activity of the lysosomal enzyme ASB. This enzyme is important for the breakdown of the glycosaminoglycans (GAGs dermatan sulfate and chondroitin sulfate, which accumulate in body tissues and organs of MPS VI patients. The storage of GAGs (especially dermatan sulfate causes bone dysplasia, joint restriction, organomegaly, heart disease, and corneal clouding, among several other problems, and reduced life span. Despite the fact that most cases are severe, there is a spectrum of severity and some cases are so attenuated that diagnosis is made late in life. Although the analysis of urinary GAGs and/or the measurement of enzyme activity in dried blood spots are useful screening methods, the diagnosis is based in the demonstration of the enzyme deficiency in leucocytes or fibroblasts, and/or in the identification of pathogenic mutations in the ARSB gene

  16. Agreement between the results of meta-analyses from case reports and from clinical studies regarding the efficacy of laronidase therapy in patients with mucopolysaccharidosis type I who initiated enzyme replacement therapy in adult age: An example of case reports meta-analyses as an useful tool for evidence-based medicine in rare diseases.

    Science.gov (United States)

    Sampayo-Cordero, Miguel; Miguel-Huguet, Bernat; Pardo-Mateos, Almudena; Moltó-Abad, Marc; Muñoz-Delgado, Cecilia; Pérez-López, Jordi

    2018-02-01

    Case reports might have a prominent role in the rare diseases field, due to the small number of patients affected by one such disease. A previous systematic review regarding the efficacy of laronidase therapy in patients with mucopolysaccharidosis type I (MPS-I) who initiated enzyme replacement therapy (ERT) in adult age has been published. The review included a meta-analysis of 19 clinical studies and the description of eleven case reports. It was of interest to perform a meta-analysis of those case reports to explore the role of such meta-analyses as a tool for evidence-based medicine in rare diseases. The study included all case reports with standard treatment regimen. Primary analysis was the percentage of case reports showing an improvement in a specific outcome. Only when that percentage was statistically higher than 5%, the improvement was confirmed as such. The outcomes that accomplished this criterion were ranked and compared to the GRADE criteria obtained by those same outcomes in the previous meta-analysis of clinical studies. There were three outcomes that had a significant improvement: Urine glycosaminoglycans, liver volume and 6-minute walking test. Positive and negative predictive values, sensitivity and specificity for the results of the meta-analysis of case reports as compared to that of clinical studies were 100%, 88.9%, 75% and 100%, respectively. Accordingly, absolute (Rho=0.82, 95%CI: 0.47 to 0.95) and relative agreement (Kappa=0.79, 95%CI: 0.593 to 0.99) between the number of case reports with improvement in a specific outcome and the GRADE evidence score for that outcome were good. Sensitivity analysis showed that agreement between the meta-analysis of case reports and that of the clinical studies were good only when using a strong confirmatory strategy for outcome improvement in case reports. We found an agreement between the results of meta-analyses from case reports and from clinical studies in the efficacy of laronidase therapy in

  17. Radiographically visualized skeletal changes associated with mucopolysaccharidosis VI in cats

    International Nuclear Information System (INIS)

    Konde, L.J.; Thrall, M.A.; Gasper, P.; Dial, S.M.; McBiles, K.; Colgan, S.; Haskins, M.

    1987-01-01

    The radiographic skeletal form and structure of all cats with mucopolysaccharidosis VI is described. Common manifestations included epiphyseal dysplasia, generalized osteoporosis, abnormal nasal turbinate development, his subluxation, impaired development of skeletal growth, pectus excavatum, hyoid hypoplasia, aplasia, hypoplasia and fragmentation or abnormal ossification of the dens, and aplasia or hypoplasia of frontal and sphenoid sinuses. The skeletal measurements of two affected cats were compared with those of normal, sex-matched littermates, and the measurements of two affected female cats were compared with those of a normal male littermate

  18. Hurler disease (mucopolysaccharidosis type IH: clinical features and consanguinity in Tunisian population

    Directory of Open Access Journals (Sweden)

    Chkioua Latifa

    2011-11-01

    Full Text Available Abstract Mucopolysaccharidosis type I (MPS I was a group of rare autosomal recessive disorder caused by the deficiency of the lysosomal enzyme, alpha -L -iduronidase, and the resulting accumulation of undergraded dematan sulfate and heparan sulfate. MPS I patients have a wide range of clinical presentations, that makes it difficult to predict patient phenotype which is needed for genetic counseling and also impedes the selection and evaluation of patients undergoing therapy bone marrow transplantation. Aim of the study consanguinity rates have been determined among 14 families with mucopolysaccharidosis type I, seen in the pediatric departments of different geographic areas of Tunisia (Central and Southern areas for the period August 2004 - August 2011 in order to investigate the relation between consanguinity and this disorder. Patients and methods Clinical and molecular analyses confirmed the diagnosis for MPS type I in the studied families. Results Most of the Tunisian MPS I patients have been identified at the homozygous status: p.P533R mutation (7 homozygous and one double heterozygous p.L578Q/p.P533R patients; 41.66% of all the investigated MPSI patients, p.F177S (1 homozygous patient; 5.55%, p.L530fs (1 patient; 5.55%, p.Y581X (2 patients; 11.11%, p.F602X (3 patients; 16.66%, p.R628X (1 patient; 5.55%. Another mutation: p.L578Q has been identified at the heterozygous status in the only double heterozygous p.L578Q/p.P533R case. Part of the mutations was the result of a founder effect. These described points are the consequences of the high rate of consanguinity. Conclusion The high frequency of p.P533R mutation could be explained by the high degree of inbreeding. This is due to the richness of the genetic background of the studied population. A multidisciplinary approach is essential to develop adequate preventive program adapted to the social, cultural, and economic context.

  19. Correction of murine mucopolysaccharidosis VII by a human. beta. -glucuronidase transgene

    Energy Technology Data Exchange (ETDEWEB)

    Kyle, J.W.; Vogler, C.; Hoffmann, J.W.; Sly, W.S. (St. Louis Univ. School of Medicine, MO (USA)); Birkenmeier, E.H.; Gwynn, B. (Jackson Laboratory, Bar Harbor, ME (USA))

    1990-05-01

    The authors recently described a murine model for mucopolysaccharidosis VII in mice that have an inherited deficiency of {beta}-glucuronidase. Affected mice, of genotype gus{sup mps}/gus{sup mps}, present clinical manifestations similar to those of humans with mucopolysaccharidosis VII (Sly syndrome) and are shown here to have secondary elevations of other lysosomal enzymes. The mucopolysaccharidosis VII phenotype in both species includes dwarfism, skeletal deformities, and premature death. Lysosome storage is visualized within enlarged vesicles and correlates biochemically with accumulation of undegraded and partially degraded glycosaminoglycans. In this report they describe the consequences of introducing the human {beta}-glucuronidase gene, GUSB, into gus{sup mps}/gus{sup mps} mice that produce virtually no murine {beta}-glucuronidase. Transgenic mice homozygous for the mucopolysaccharidosis VII mutation expressed high levels of human {beta}-glucuronidase activity in all tissues examined and were phenotypically normal. Biochemically, both the intralysosomal storage of glycosaminoglycans and the secondary elevation of other acid hydrolases were corrected. These findings demonstrate that the GUSB transgene is expressed in gus{sup mps}/gus{sup mps} mice and that human {beta}-glucuronidase corrects the murine mucopolysaccharidosis storage disease.

  20. Mucopolysaccharidosis type II: European recommendations for the diagnosis and multidisciplinary management of a rare disease

    DEFF Research Database (Denmark)

    Scarpa, Maurizio; Almássy, Zsuzsanna; Beck, Michael

    2011-01-01

    Mucopolysaccharidosis type II (MPS II) is a rare, life-limiting, X-linked recessive disease characterised by deficiency of the lysosomal enzyme iduronate-2-sulfatase. Consequent accumulation of glycosaminoglycans leads to pathological changes in multiple body systems. Age at onset, signs and symp......Mucopolysaccharidosis type II (MPS II) is a rare, life-limiting, X-linked recessive disease characterised by deficiency of the lysosomal enzyme iduronate-2-sulfatase. Consequent accumulation of glycosaminoglycans leads to pathological changes in multiple body systems. Age at onset, signs...... paediatricians, specialist nurses, otorhinolaryngologists, orthopaedic surgeons, ophthalmologists, cardiologists, pneumologists, anaesthesiologists, neurologists, physiotherapists, occupational therapists, speech therapists, psychologists, social workers, homecare companies and patient societies. Take...

  1. Distribution of Heparan Sulfate Oligosaccharides in Murine Mucopolysaccharidosis Type IIIA

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    Kerryn Mason

    2014-12-01

    Full Text Available Heparan sulfate (HS catabolism begins with endo-degradation of the polysaccharide to smaller HS oligosaccharides, followed by the sequential action of exo-enzymes to reduce these oligosaccharides to monosaccharides and inorganic sulfate. In mucopolysaccharidosis type IIIA (MPS IIIA the exo-enzyme, N-sulfoglucosamine sulfohydrolase, is deficient resulting in an inability to hydrolyze non-reducing end glucosamine N-sulfate esters. Consequently, partially degraded HS oligosaccharides with non-reducing end glucosamine sulfate esters accumulate. We investigated the distribution of these HS oligosaccharides in tissues of a mouse model of MPS IIIA using high performance liquid chromatography electrospray ionization-tandem mass spectrometry. Oligosaccharide levels were compared to total uronic acid (UA, which was used as a measure of total glycosaminoglycan. Ten oligosaccharides, ranging in size from di- to hexasaccharides, were present in all the tissues examined including brain, spleen, lung, heart, liver, kidney and urine. However, the relative levels varied up to 10-fold, suggesting different levels of HS turnover and storage. The relationship between the di- and tetrasaccharides and total UA was tissue specific with spleen and kidney showing a different disaccharide:total UA ratio than the other tissues. The hexasaccharides showed a stronger correlation with total UA in all tissue types suggesting that hexasaccharides may more accurately reflect the storage burden in these tissues.

  2. Mediastinal Tracheostoma for Treatment of Tracheostenosis after Tracheostomy in a Patient with Mucopolysaccharidosis-Induced Tracheomalacia

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    Yasuhiro Chikaishi

    2017-01-01

    Full Text Available Background. Treatment of tracheostenosis after tracheostomy in pediatric patients is often difficult. Mucopolysaccharidosis is a lysosomal storage disease that may induce obstruction of the airways. Case Presentation. A 16-year-old male patient underwent long-term follow-up after postnatal diagnosis of type II mucopolysaccharidosis. At 11 years of age, tracheostomy was performed for mucopolysaccharidosis-induced laryngeal stenosis. One week prior to presentation, he was admitted to another hospital on an emergency basis for major dyspnea. He was diagnosed with tracheostenosis caused by granulation. The patient was then referred to our institution. The peripheral view of his airway was difficult because of mucopolysaccharidosis-induced tracheomalacia. For airway management, a mediastinal tracheostoma was created with extracorporeal membrane oxygenation. To maintain the blood flow, the skin incision for the mediastinal tracheal hole was sharply cut without an electrotome. The postoperative course was uneventful, and the patient was weaned from the ventilator on postoperative day 19. He was discharged 1.5 months postoperatively. Although he was referred to another institution because of respiratory failure caused by his primary disease 6 months postoperatively, his airway management remained successful for 1.5 years postoperatively. Conclusion. Mediastinal tracheostomy was useful for treatment of tracheostenosis caused by granulation tissue formation after a tracheostomy.

  3. Processing of mutant N-acetyl-alpha-glucosaminidase in mucopolysaccharidosis type IIIB fibroblasts cultured at low temperature

    NARCIS (Netherlands)

    Meijer, O. L. M.; te Brinke, H.; Ofman, R.; IJlst, L.; Wijburg, F. A.; van Vlies, N.

    2017-01-01

    Background: The autosomal recessive, neurodegenerative disorder mucopolysaccharidosis type IIIB (MPSIIIB) is caused by a deficiency of the lysosomal enzyme N-acetyl-alpha-glucosaminidase (NAGLU), resulting in accumulation of heparan sulfate. The disease spectrum comprises a severe, rapidly

  4. Mucopolysaccharidosis type VI in a juvenile miniature schnauzer dog with concurrent hypertriglyceridemia, necrotizing pancreatitis, and diabetic ketoacidosis.

    Science.gov (United States)

    Pérez, Mayrim L; Kridel, Heather A; Gallagher, Alex; Sheppard, Barbara J; Reese, Shona; Kondo, Hirotaka; Alleman, Rick; Giger, Urs

    2015-03-01

    A 7-month-old, neutered male miniature schnauzer dog with a history of cryptorchidism and umbilical hernia was referred for diabetic ketoacidosis. Clinical evaluation revealed stunted growth, skeletal abnormalities, hypertriglyceridemia, diabetic ketoacidosis, and acute necrotizing pancreatitis. Further testing was diagnostic for mucopolysaccharidosis type VI causing the stunted growth and skeletal deformities, but no connection between mucopolysaccharidosis type VI, hypertriglyceridemia, and pancreatic diseases was found.

  5. Syringomyelia in mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): imaging findings following bone marrow transplantation

    International Nuclear Information System (INIS)

    Hite, S.H.; Krivit, W.; Haines, S.J.; Whitley, C.B.

    1997-01-01

    We present the imaging findings in a patient with mucopolysaccharidosis (MPS) type VI (Maroteaux-Lamy syndrome) who developed holocord syringomyelia. This represents the only reported case of syrinx formation in a child with MPS VI. Clinical, neurologic and spinal magnetic resonance imaging findings are presented. The patient has maintained a stable clinical and neurologic course over the period following allogeneic bone marrow transplant. (orig.). With 3 figs

  6. Structure of sulfamidase provides insight into the molecular pathology of mucopolysaccharidosis IIIA

    International Nuclear Information System (INIS)

    Sidhu, Navdeep S.; Schreiber, Kathrin; Pröpper, Kevin; Becker, Stefan; Usón, Isabel; Sheldrick, George M.; Gärtner, Jutta; Krätzner, Ralph; Steinfeld, Robert

    2014-01-01

    Mucopolysaccharidosis IIIA is a fatal neurodegenerative disease that typically manifests itself in childhood and is caused by mutations in the gene for the lysosomal enzyme sulfamidase. The first structure of this enzyme is presented, which provides insight into the molecular basis of disease-causing mutations, and the enzymatic mechanism is proposed. Mucopolysaccharidosis type IIIA (Sanfilippo A syndrome), a fatal childhood-onset neurodegenerative disease with mild facial, visceral and skeletal abnormalities, is caused by an inherited deficiency of the enzyme N-sulfoglucosamine sulfohydrolase (SGSH; sulfamidase). More than 100 mutations in the SGSH gene have been found to reduce or eliminate its enzymatic activity. However, the molecular understanding of the effect of these mutations has been confined by a lack of structural data for this enzyme. Here, the crystal structure of glycosylated SGSH is presented at 2 Å resolution. Despite the low sequence identity between this unique N-sulfatase and the group of O-sulfatases, they share a similar overall fold and active-site architecture, including a catalytic formylglycine, a divalent metal-binding site and a sulfate-binding site. However, a highly conserved lysine in O-sulfatases is replaced in SGSH by an arginine (Arg282) that is positioned to bind the N-linked sulfate substrate. The structure also provides insight into the diverse effects of pathogenic mutations on SGSH function in mucopolysaccharidosis type IIIA and convincing evidence for the molecular consequences of many missense mutations. Further, the molecular characterization of SGSH mutations will lay the groundwork for the development of structure-based drug design for this devastating neurodegenerative disorder

  7. Structure of sulfamidase provides insight into the molecular pathology of mucopolysaccharidosis IIIA

    Energy Technology Data Exchange (ETDEWEB)

    Sidhu, Navdeep S. [University of Göttingen, Robert-Koch-Strasse 40, 37075 Göttingen (Germany); University of Göttingen, Tammannstrasse 4, 37077 Göttingen (Germany); Schreiber, Kathrin [University of Göttingen, Robert-Koch-Strasse 40, 37075 Göttingen (Germany); Pröpper, Kevin [University of Göttingen, Tammannstrasse 4, 37077 Göttingen (Germany); Becker, Stefan [Max Planck Institute for Biophysical Chemistry, Am Fassberg 11, 37077 Göttingen (Germany); Usón, Isabel [Instituto de Biologia Molecular de Barcelona (IBMB–CSIC), Barcelona Science Park, Baldiri Reixach 15, 08028 Barcelona (Spain); Institucio Catalana de Recerca i Estudis Avancats (ICREA), (Spain); Sheldrick, George M. [University of Göttingen, Tammannstrasse 4, 37077 Göttingen (Germany); Gärtner, Jutta; Krätzner, Ralph, E-mail: rkraetz@gwdg.de; Steinfeld, Robert, E-mail: rkraetz@gwdg.de [University of Göttingen, Robert-Koch-Strasse 40, 37075 Göttingen (Germany)

    2014-05-01

    Mucopolysaccharidosis IIIA is a fatal neurodegenerative disease that typically manifests itself in childhood and is caused by mutations in the gene for the lysosomal enzyme sulfamidase. The first structure of this enzyme is presented, which provides insight into the molecular basis of disease-causing mutations, and the enzymatic mechanism is proposed. Mucopolysaccharidosis type IIIA (Sanfilippo A syndrome), a fatal childhood-onset neurodegenerative disease with mild facial, visceral and skeletal abnormalities, is caused by an inherited deficiency of the enzyme N-sulfoglucosamine sulfohydrolase (SGSH; sulfamidase). More than 100 mutations in the SGSH gene have been found to reduce or eliminate its enzymatic activity. However, the molecular understanding of the effect of these mutations has been confined by a lack of structural data for this enzyme. Here, the crystal structure of glycosylated SGSH is presented at 2 Å resolution. Despite the low sequence identity between this unique N-sulfatase and the group of O-sulfatases, they share a similar overall fold and active-site architecture, including a catalytic formylglycine, a divalent metal-binding site and a sulfate-binding site. However, a highly conserved lysine in O-sulfatases is replaced in SGSH by an arginine (Arg282) that is positioned to bind the N-linked sulfate substrate. The structure also provides insight into the diverse effects of pathogenic mutations on SGSH function in mucopolysaccharidosis type IIIA and convincing evidence for the molecular consequences of many missense mutations. Further, the molecular characterization of SGSH mutations will lay the groundwork for the development of structure-based drug design for this devastating neurodegenerative disorder.

  8. Resolution of Hydronephrosis in a Patient With Mucopolysaccharidosis Type II With Enzyme Replacement Therapy.

    Science.gov (United States)

    Nishiyama, Kei; Imai, Takashi; Ohkubo, Kazuhiro; Sanefuji, Masafumi; Takada, Hidetoshi

    2017-03-01

    Mucopolysaccharidosis type II (MPS II) is caused by deficiency of lysosomal enzyme iduronate-2-sulfatase. Insufficient activity of the enzyme results in accumulation of glycosaminoglycans leading to progressive multisystem pathologies. MPS II is less likely to be complicated by kidney and urinary tract problems. We report a boy with MPS II, who developed left hydronephrosis. His hydronephrosis improved after starting enzyme replacement therapy. It was suggested that MPS II was closely associated with the pathogenesis of hydronephrosis. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Early Umbilical Cord Blood-Derived Stem Cell Transplantation Does Not Prevent Neurological Deterioration in Mucopolysaccharidosis Type III

    NARCIS (Netherlands)

    Welling, Lindsey; Marchal, Jan Pieter; van Hasselt, Peter; van der Ploeg, Ans T; Wijburg, Frits A; Boelens, Jaap Jan

    2015-01-01

    Mucopolysaccharidosis type III (MPS III), or Sanfilippo disease, is a neurodegenerative lysosomal storage disease (LSD) caused by defective lysosomal degradation of heparan sulfate (HS). No effective disease-modifying therapy is yet available. In contrast to some other neuronopathic LSDs, bone

  10. Development of idursulfase therapy for mucopolysaccharidosis type II (Hunter syndrome: the past, the present and the future

    Directory of Open Access Journals (Sweden)

    Whiteman DAH

    2017-08-01

    Full Text Available David AH Whiteman,* Alan Kimura* Research & Development, Shire Human Genetic Therapies, Inc., Lexington, MA, USA *These authors contributed equally to this work Abstract: Mucopolysaccharidosis type II (MPS II; Hunter syndrome; OMIM 309900 is a rare, multisystemic, progressive lysosomal storage disease caused by deficient activity of the iduronate-2-sulfatase (I2S enzyme. Accumulation of the glycosaminoglycans dermatan sulfate and heparan sulfate results in a broad range of disease manifestations that are highly variable in presentation and severity; notably, approximately two-thirds of individuals are affected by progressive central nervous system involvement. Historically, management of this disease was palliative; however, during the 1990s, I2S was purified to homogeneity for the first time, leading to cloning of the corresponding gene and offering a means of addressing the underlying cause of MPS II using enzyme replacement therapy (ERT. Recombinant I2S (idursulfase was produced for ERT using a human cell line and was shown to be indistinguishable from endogenous I2S. Preclinical studies utilizing the intravenous route of administration provided valuable insights that informed the design of the subsequent clinical studies. The pivotal Phase II/III clinical trial of intravenous idursulfase (Elaprase®; Shire, Lexington, MA, USA demonstrated improvements in a range of clinical parameters; based on these findings, intravenous idursulfase was approved for use in patients with MPS II in the USA in 2006 and in Europe and Japan in 2007. Evidence gained from post-approval programs has helped to improve our knowledge and understanding of management of patients with the disease; as a result, idursulfase is now available to young pediatric patients, and in some countries patients have the option to receive their infusions at home. Although ERT with idursulfase has been shown to improve somatic signs and symptoms of MPS II, the drug does not cross the

  11. Female Mucopolysaccharidosis IIIA Mice Exhibit Hyperactivity and a Reduced Sense of Danger in the Open Field Test

    OpenAIRE

    Langford-Smith, Alex; Langford-Smith, Kia J.; Jones, Simon A.; Wynn, Robert F.; Wraith, J. E.; Wilkinson, Fiona L.; Bigger, Brian W.

    2011-01-01

    Reliable behavioural tests in animal models of neurodegenerative diseases allow us to study the natural history of disease and evaluate the efficacy of novel therapies. Mucopolysaccharidosis IIIA (MPS IIIA or Sanfilippo A), is a severe, neurodegenerative lysosomal storage disorder caused by a deficiency in the heparan sulphate catabolising enzyme, sulfamidase. Undegraded heparan sulphate accumulates, resulting in lysosomal enlargement and cellular dysfunction. Patients suffer a progressive lo...

  12. Determination of genotypic and clinical characteristics of Colombian patients with mucopolysaccharidosis IVA

    Directory of Open Access Journals (Sweden)

    Tapiero-Rodriguez SM

    2018-04-01

    Full Text Available Sandra M Tapiero-Rodriguez,1 Johanna C Acosta Guio,1 Gloria Liliana Porras-Hurtado,2 Natalia García,3 Martha Solano,4 Harry Pachajoa,5 Harvy M Velasco1 1Universidad Nacional de Colombia, Departamento de morfología, Maestría de genética humana, Bogotá, 2Family Compensation Fund of Risaralda, Pereira, 3Faculty of Medicine, Manizales University, Manizales, 4Department of Neuropediatrics, Cardioinfantil Foundation, Bogotá, 5Centro de Investigaciones en Anomalías Congénitas y Enfermedades Raras, Universidad ICESI y Fundación Valle del Lili, Cali, Colombia Background: As mucopolysaccharidosis IVA (MPS IVA is the most frequent MPS in Colombia, this paper aims to describe its clinical and mutational characteristics in 32 diagnosed patients included in this study. Methods: Genotyping was completed by amplification and Sanger sequencing of the GALNS gene. The SWISS-model platform was used for bioinformatic analysis, and mutant proteins were generated by homology from the wild-type GALNS code 4FDI template from the Protein Data Bank (PDB database. Docking was performed using the GalNAc6S ligand (PubChem CID: 193456 by AutoDock Vina 1.0 and visualized in PyMOL and LigPlot+. Results: Eleven variants were identified, and one new pathogenic variant was described in the heterozygous state, which is consistent with genotype c. 319 G>T or p.Ala107Ser. The pathogenic variant c.901G>T or p.Gly301Cys was the most frequent mutation with 51.6% of alleles. Docking revealed affinity energy of −5.9 Kcal/mol between wild-type GALNS and the G6S ligand. Some changes were evidenced at the intermolecular interaction level, and affinity energy for each mutant decreased. Conclusion: Clinical variables and genotypic analysis were similar to those reported for other world populations. Genotypic data showed greater allelic heterogeneity than those previously reported. Bioinformatics tools showed differences in the binding interactions of mutant proteins with the G6S

  13. The Effect of Neonatal Gene Therapy on Skeletal Manifestations in Mucopolysaccharidosis VII Dogs after a Decade

    Science.gov (United States)

    Xing, Elizabeth M.; Knox, Van W.; O'Donnell, Patricia A.; Sikura, Tracey; Liu, Yuli; Wu, Susan; Casal, Margret L.; Haskins, Mark E.; Ponder, Katherine P.

    2013-01-01

    Mucopolysaccharidosis (MPS) VII is a lysosomal storage disease due to deficient activity of β-glucuronidase (GUSB), and results in glycosaminoglycan accumulation. Skeletal manifestations include bone dysplasia, degenerative joint disease, and growth retardation. One gene therapy approach for MPS VII involves neonatal intravenous injection of a gamma retroviral vector expressing GUSB, which results in stable expression in liver and secretion of enzyme into blood at levels predicted to be similar or higher to enzyme replacement therapy. The goal of this study was to evaluate the long-term effect of neonatal gene therapy on skeletal manifestations in MPS VII dogs. Treated MPS VII dogs could walk throughout their lives, while untreated MPS VII dogs could not stand beyond 6 months and were dead by 2 years. Luxation of the coxofemoral joint and the patella, dysplasia of the acetabulum and supracondylar ridge, deep erosions of the distal femur, and synovial hyperplasia were reduced, and the quality of articular bone was improved in treated dogs at 6 to 11 years of age compared with untreated MPS VII dogs at 2 years or less. However, treated dogs continued to have osteophyte formation, cartilage abnormalities, and an abnormal gait. Enzyme activity was found near synovial blood vessels, and there was 2% as much GUSB activity in synovial fluid as in serum. We conclude that neonatal gene therapy reduces skeletal abnormalities in MPS VII dogs, but clinically-relevant abnormalities remain. Enzyme replacement therapy will probably have similar limitations long-term. PMID:23628461

  14. [The thesis of judicialization of health care by the elites: medication for mucopolysaccharidosis].

    Science.gov (United States)

    Medeiros, Marcelo; Diniz, Debora; Schwartz, Ida Vanessa Doederlein

    2013-04-01

    This paper evaluates the hypothesis that the judicialization of medicine for mucopolysaccharidosis in Brazil is an action promoted by economic elites. Previous studies upholding the thesis of judicialization by elites in the case of other types of medication that are more costly for the Unified Health Service are discussed. An analysis of all 196 processes containing information about judicial processes brought to court between February 2006 and December 2010 that ended by determining that the State should provide such medication free of charge to patients was conducted. There is evidence that attorneys' fees were covered by entities interested in the results of judicialization, such as the distributors or pharmaceutical industries. Patients may also be migrating for diagnosis and treatment to university centers that are a benchmark for medical innovation in the country, as the option for public health services is related to their higher technical and scientific capacity. Therefore, the resort to private lawyers, indicators of social exclusion based on the address of patients and the use of public health services, are not adequate class information to corroborate or refute the thesis of judicialization by the elites.

  15. Oxidative profile exhibited by Mucopolysaccharidosis type IVA patients at diagnosis: Increased keratan urinary levels

    Directory of Open Access Journals (Sweden)

    Bruna Donida

    2017-06-01

    Full Text Available Morquio A disease (Mucopolysaccharidosis type IVA, MPS IVA is one of the 11 mucopolysaccharidoses (MPSs, a heterogeneous group of inherited lysosomal storage disorders (LSDs caused by deficiency in enzymes need to degrade glycosaminoglycans (GAGs. Morquio A is characterized by a decrease in N-acetylgalactosamine-6-sulfatase activity and subsequent accumulation of keratan sulfate and chondroitin 6-sulfate in cells and body fluids. As the pathophysiology of this LSD is not completely understood and considering the previous results of our group concerning oxidative stress in Morquio A patients receiving enzyme replacement therapy (ERT, the aim of this study was to investigate oxidative stress parameters in Morquio A patients at diagnosis. It was studied 15 untreated Morquio A patients, compared with healthy individuals. The affected individuals presented higher lipid peroxidation, assessed by urinary 15-F2t-isoprostane levels and no protein damage, determined by sulfhydryl groups in plasma and di-tyrosine levels in urine. Furthermore, Morquio A patients showed DNA oxidative damage in both pyrimidines and purines bases, being the DNA damage positively correlated with lipid peroxidation. In relation to antioxidant defenses, affected patients presented higher levels of reduced glutathione (GSH and increased activity of glutathione peroxidase (GPx, while superoxide dismutase (SOD and glutathione reductase (GR activities were similar to controls. Our findings indicate that Morquio A patients present at diagnosis redox imbalance and oxidative damage to lipids and DNA, reinforcing the idea about the importance of antioxidant therapy as adjuvant to ERT, in this disorder.

  16. Mucopolysaccharidosis IVA: Four new exonic mutations in patients with N-acetylgalactosamine-6-sulfate sulfatase deficiency

    Energy Technology Data Exchange (ETDEWEB)

    Tomatsu, Shunji; Fukuda, Seiji; Yamagishi, Atsushi [Gifu Univ. (Japan)] [and others

    1996-05-01

    We report four new mutations in Japanese patients with mucopolysaccharidosis IVA (MPSIVA) who were heterozygous for a common double gene deletion. A nonsense mutation of CAG to TAG at codon 148 in exon 4 was identified, resulting in a change of Q to a stop codon and three missense mutations: V (GTC) to A (GCC) at codon 138 in exon 4, P (CCC) to S (TCC) at codon 151 in exon 5, and P (CCC) to L (CTC) at codon 151 in exon 5. Introduction of these mutations into the normal GALNS cDNA and transient expression in cultured fibroblasts resulted in a significant decrease in the enzyme activity. V138A and Q148X mutations result in changes of restriction site, which were analyzed by restriction-enzyme assay. P151S and P151L mutations that did not alter the restriction site were detected by direct sequencing or allele specific oligohybridization. Detection of the double gene deletion was initially done using Southern blots and was confirmed by PCR. Haplotypes were determined using seven polymorphisms to the GALNS locus in families with the double gene deletion. Haplotype analysis showed that the common double gene deletion occurred on a single haplotype, except for some variation in a VNTR-like polymorphism. This finding is consistent with a common founder for all individuals with this mutation. 48 refs., 5 figs., 1 tab.

  17. Progressive neurologic and somatic disease in a novel mouse model of human mucopolysaccharidosis type IIIC

    Directory of Open Access Journals (Sweden)

    Sara Marcó

    2016-09-01

    Full Text Available Mucopolysaccharidosis type IIIC (MPSIIIC is a severe lysosomal storage disease caused by deficiency in activity of the transmembrane enzyme heparan-α-glucosaminide N-acetyltransferase (HGSNAT that catalyses the N-acetylation of α-glucosamine residues of heparan sulfate. Enzyme deficiency causes abnormal substrate accumulation in lysosomes, leading to progressive and severe neurodegeneration, somatic pathology and early death. There is no cure for MPSIIIC, and development of new therapies is challenging because of the unfeasibility of cross-correction. In this study, we generated a new mouse model of MPSIIIC by targeted disruption of the Hgsnat gene. Successful targeting left LacZ expression under control of the Hgsnat promoter, allowing investigation into sites of endogenous expression, which was particularly prominent in the CNS, but was also detectable in peripheral organs. Signs of CNS storage pathology, including glycosaminoglycan accumulation, lysosomal distension, lysosomal dysfunction and neuroinflammation were detected in 2-month-old animals and progressed with age. Glycosaminoglycan accumulation and ultrastructural changes were also observed in most somatic organs, but lysosomal pathology seemed most severe in liver. Furthermore, HGSNAT-deficient mice had altered locomotor and exploratory activity and shortened lifespan. Hence, this animal model recapitulates human MPSIIIC and provides a useful tool for the study of disease physiopathology and the development of new therapeutic approaches.

  18. Open field locomotor activity and anxiety-related behaviors in mucopolysaccharidosis type IIIA mice.

    Science.gov (United States)

    Lau, Adeline A; Crawley, Allison C; Hopwood, John J; Hemsley, Kim M

    2008-08-05

    Mucopolysaccharidosis (MPS) IIIA, or Sanfilippo syndrome, is a lysosomal storage disorder characterized by severe and progressive neuropathology. Following an asymptomatic period, patients may present with sleep disturbances, cognitive decline, aggressive tendencies and hyperactivity. A naturally-occurring mouse model of MPS IIIA also exhibits many of these behavioral features and has been recently back-crossed onto a C57BL/6 genetic background. To more thoroughly characterize the behavioral phenotype of congenic MPS IIIA mice, we assessed exploratory activity and unconditioned anxiety-related behavior in the elevated plus maze (EPM) and open field locomotor activity. Although MPS IIIA male mice were less active in the EPM at 18 and 20 weeks of age, they were more likely to explore the open arms than their normal counter-parts suggesting reduced anxiety. Repeated EPM testing reduced exploration of the open arms in MPS IIIA mice. In the open field test, significant reductions in activity were evident in naïve-tested male MPS IIIA mice from 10 weeks of age. Female normal and MPS IIIA mice displayed similar exploratory activity in the open field test. These differences in anxiety and locomotor activity will allow us to evaluate the efficacy of therapeutic regimes for MPS IIIA as a forerunner to developing safe and effective therapies for Sanfilippo patients.

  19. [Consequences of the judicialization of health policies: the cost of medicines for mucopolysaccharidosis].

    Science.gov (United States)

    Diniz, Debora; Medeiros, Marcelo; Schwartz, Ida Vanessa D

    2012-03-01

    This study analyzes expenditures backed by court rulings to ensure the public provision of medicines for treatment of mucopolysaccharidosis (MPS), a rare disease that requires high-cost drugs not covered by the Brazilian government's policy for pharmaceutical care and which have disputed clinical efficacy. The methodology included a review of files from 196 court rulings ordering the Brazilian Ministry of Health to provide the medicines, in addition to Ministry of Health administrative records. According to the analysis, the "judicialization" of the health system subjected the Brazilian government to a monopoly in the distribution of medicines and consequently the loss of its capacity to manage drug purchases. The study also indicates that the imposition of immediate, individualized purchases prevents obtaining economies of scale with planned procurement of larger amounts of the medication, besides causing logistic difficulties in controlling the amounts consumed and stored. In conclusion, litigation results from the lack of a clear policy in the health system for rare diseases in general, thereby leading to excessive expenditures for MPS treatment.

  20. Corrections of diverse forms of lower limb deformities in patients with mucopolysaccharidosis type IVA (Morquio syndrome

    Directory of Open Access Journals (Sweden)

    Ali Al Kaissi

    2016-01-01

    Full Text Available Background: Thoracolumbar kyphosis has been considered as the first presenting deformity and is often a key diagnostic clue noted in children with mucopolysaccharidosis (MPS type IV (Morquio′s syndrome. However, we observed that the progressive irregularities of the epiphyses of the long bones were the most prominent skeletal pathology, causing effectively the development of diverse forms of lower limbs deformities with extreme variation in age of onset. Materials and Methods: Ten patients (seven children and three adults with an average age of 15 years have been enrolled in this study. Age of diagnosis of MPS IVA has a variable age of onset and a MISLEADING rate of severity. Hip dislocations, genu valgum, protrusio acetabuli and osteoarthritis were the most common lower limbs deformities in these patients. Clinical and radiographic phenotypes were the baseline tools of documentation. Urinary screening and genotypic characterizations have been applied accordingly. Results: Combined pelvic and femoral procedures for hip dislocation, epiphysiodeses and supracondylar osteotomy for genu valgum and hip arthroplasty for protrusio acetabuli have been performed. All patients manifested insufficient activity of N-acetylgalactosamine-6-sulphate sulphatase, an enzyme that degrades keratin sulphate and chondroitin-6 sulphate. Conclusion: The extensive clinical heterogeneity contributed significantly in the delay in establishing the diagnosis particularly in adult patients with MPS IV. The epiphyseal irregularities of the long bones and the progressive flattening pathology of MPS IV A were the reason to falsely diagnose some patients as spondyloepiphyseal dysplasia congenital and/or tarda. Proximal femoral osteotomy, realignment osteotomy and total hip arthroplasty have been performed for coxa vara, genu valgum and protrusio acetabuli, respectively, in children and adult group of patients. The importance of early diagnosis on MPS IV A is to receive enzyme

  1. [The progressive reduction of functioning in the course of mucopolysaccharidosis type IIIA - longitudinal study of two siblings].

    Science.gov (United States)

    Michalska, Agata; Nawrocka, Małgorzata; Znój, Dorota

    2013-01-01

    This paper presents a description of changes in the functioning of two siblings diagnosed with mucopolysaccharidosis type III A. Both are under specialist care exercised by the Rehabilitation, Care and Education Centre in the city Kielce, including care of a oligophrenopedagogue, a psychologist, a speech therapist and a physiotherapist. Evaluation of changes in functioning of two siblings diagnosed with mucopolysaccharidosis type IIIA. The longitudinal study covered two children with MPS type IIIA. During the 29 months of observation, there were six measurements on the basis of PPAC Gunzburg Inventory in the Polish adaptation by Tadeusz Witkowski. The results are shown in the form of PPAC diagrams and profiles of functioning. Despite the differences in the presence and severity of somatic and neurocognitive symptoms, functioning both of the boy and the girl does not differ from functioning described in the literature. Therapeutic interventions have produced short-term improvements in its area of self-service, communication and activities. Despite the similar trend of changes in functioning, there is an inter-individual variability in the quality of patterns and dynamics of progress. The progressive decrease in the level of functioning in patients with MPS IIIA does not preclude the acquisition of new skills. They are not permanent, however. There is a need for functional assessment in order to learn more about the specificity of the disease and to assume an individualised therapeutic approach aimed at improving the quality of life of patients with MPS IIIA and, indirectly, the quality of life of their families.

  2. Heart and Cardiovascular Involvement in Patients with Mucopolysaccharidosis Type IVA (Morquio-A Syndrome.

    Directory of Open Access Journals (Sweden)

    Christoph Kampmann

    Full Text Available Mucopolysaccharidosis (MPS IVA is a rare lysosomal storage disorder with multiple skeletal and non-skeletal abnormalities requiring multiple surgical interventions. It is well known that patients with MPS IVA suffer from tachycardia, but cardiac and hemodynamic alterations have not been reported to date. We investigated the cardiovascular and hemodynamic alterations in patients with MPS IVA and developed a possible patho-mechanism for cardiovascular deterioration during anesthesia.In this observational study, serial cardiac examinations were performed in 54 patients with MPS IVA who were followed at the Children's Hospital of the Mainz Medical University (Mainz, Germany between 1991 and 2014 (follow-up 1-24 years; median 5.8 years. Results were compared with data from a large central European cohort of more than 2000 healthy infants and children.None of the patients had arterial hypertension, but 4% had evidence of increased pulmonary artery pressure. Patients developed aortic root extension up to 6.9 standard deviations above normal. Left-sided valve leaflet thickening occurred in 26 patients (five with valve disease. Patients had lower left ventricular dimensions (z: -1.02±0.1, lower stroke volumes (z: -2.3±0.17, lower left ventricular mass (z: -1.5±0.21, but higher wall thickness (z: +0.8±0.16, and higher work index (z: +2.5±0.2 compared to healthy control subjects. Cardiac output was preserved by an increase in heart rate of 21%. Sixty % of patients showed impaired diastolic filling; heart rate (99.0±1.8 vs. 92.0±2.1 bpm, age (18.0±1.8 vs. 14.2±1 years, and cardiothoracic ratio (61.6±3.6% vs. 55±4.2% of these patients were higher compared to those with normal filling.The results of this study suggest an age-progressive disproportion of the intra-thoracic organs of patients with MPS IVA, which is accompanied by aortic root extension and thickened left ventricles, with reduced stroke volumes, impaired diastolic filling patterns, and

  3. Intracerebral gene therapy in children with mucopolysaccharidosis type IIIB syndrome: an uncontrolled phase 1/2 clinical trial.

    Science.gov (United States)

    Tardieu, Marc; Zérah, Michel; Gougeon, Marie-Lise; Ausseil, Jérome; de Bournonville, Stéphanie; Husson, Béatrice; Zafeiriou, Dimitrios; Parenti, Giancarlo; Bourget, Philippe; Poirier, Béatrice; Furlan, Valérie; Artaud, Cécile; Baugnon, Thomas; Roujeau, Thomas; Crystal, Ronald G; Meyer, Christian; Deiva, Kumaran; Heard, Jean-Michel

    2017-09-01

    Mucopolysaccharidosis type IIIB syndrome (also known as Sanfilippo type B syndrome) is a lysosomal storage disease resulting in progressive deterioration of cognitive acquisition after age 2-4 years. No treatment is available for the neurological manifestations of the disease. We sought to assess the safety and efficacy of a novel intracerebral gene therapy. Local regulatory authorities in France allowed inclusion of up to four children in this phase 1/2 study. Treatment was 16 intraparenchymal deposits (four in the cerebellum) of a recombinant adenoassociated viral vector serotype 2/5 (rAAV2/5) encoding human α-N-acetylglucosaminidase (NAGLU) plus immunosuppressive therapy. We assessed tolerance, neurocognitive progression, brain growth, NAGLU enzymatic activity in CSF, and specific anti-NAGLU immune response for 30 months after surgery. This trial is registered with EudraCT, number 2012-000856-33, and the International Standard Clinical Trial Registry, number ISRCTN19853672. Of seven eligible children, the four youngest, from France (n=2), Italy (n=1), and Greece (n=1), aged 20, 26, 30, and 53 months, were included between February, 2012, and February, 2014. 125 adverse events were recorded, of which 117 were treatment emergent and included six classified as severe, but no suspected unexpected serious adverse drug reactions were seen. Vector genomes were detected in blood for 2 days after surgery. Compared with the natural history of mucopolysaccharidosis type III syndromes, neurocognitive progression was improved in all patients, with the youngest patient having function close to that in healthy children. Decrease in developmental quotient was -11·0 points in patient one, -23·0 in patient two, -29·0 in patient three, and -17·0 in patient four, compared with -37·7 in the natural history of the disease. NAGLU activity was detected in lumbar CSF and was 15-20% of that in unaffected children. Circulating T lymphocytes that proliferated and produced tumour

  4. Restoration of central nervous system alpha-N-acetylglucosaminidase activity and therapeutic benefits in mucopolysaccharidosis IIIB mice by a single intracisternal recombinant adeno-associated viral type 2 vector delivery.

    Science.gov (United States)

    Fu, Haiyan; DiRosario, Julianne; Kang, Lu; Muenzer, Joseph; McCarty, Douglas M

    2010-07-01

    Finding efficient central nervous system (CNS) delivery approaches has been the major challenge facing therapeutic development for treating diseases with global neurological manifestation, such as mucopolysaccharidosis (MPS) IIIB, a lysosomal storage disease, caused by autosomal recessive defect of alpha-N-acetylglucosaminidase (NaGlu). Previously, we developed an approach, intracisternal (i.c.) injection, to deliver recombinant adeno-associated viral (rAAV) vector to the CNS of mice, leading to a widespread periventricular distribution of transduction. In the present study, we delivered rAAV2 vector expressing human NaGlu into the CNS of MPS IIIB mice by an i.c. injection approach, to test its therapeutic efficacy and feasibility for treating the neurological manifestation of the disease. We demonstrated significant functional neurological benefits of a single i.c. vector infusion in adult MPS IIIB mice. The treatment slowed the disease progression by mediating widespread recombinant NaGlu expression in the CNS, resulting in the reduction of brain lysosomal storage pathology, significantly improved cognitive function and prolonged survival. However, persisting motor function deficits suggested that pathology in areas outside the CNS contributes to the MPS IIIB behavioral phenotype. The therapeutic benefit of i.c. rAAV2 delivery was dose-dependent and could be attribute solely to the CNS transduction because the procedure did not lead to detectable transduction in somatic tissues. A single IC rAAV2 gene delivery is functionally beneficial for treating the CNS disease of MPS IIIB in mice. It is immediately clinically translatable, with the potential of improving the quality of life for patients with MPS IIIB.

  5. Early treatment with laronidase improves clinical outcomes in patients with attenuated MPS I: a retrospective case series analysis of nine sibships

    OpenAIRE

    Al-Sannaa, Nouriya A.; Bay, Luisa; Barbouth, Deborah S.; Benhayoun, Youssef; Goizet, Cyril; Guelbert, Norberto; Jones, Simon A.; Kyosen, Sandra Obikawa; Martins, Ana Maria; Phornphutkul, Chanika; Reig, Celia; Pleat, Rebecca; Fallet, Shari; Ivanovska Holder, Iva

    2015-01-01

    Background Enzyme replacement therapy (ERT) with laronidase, (recombinant human α-L-iduronidase; Aldurazyme) is the primary treatment option for patients with attenuated mucopolysaccharidosis type I (MPS I). This study examined the effect of early ERT on clinical manifestations. Methods This multinational, retrospective case series abstracted data from records of 20 patients with Hurler-Scheie syndrome within nine sibships that included older siblings treated with laronidase after the develop...

  6. Gasto energético na marcha em pacientes com mucopolissacaridose Energy expenditure during gait in patients with mucopolysaccharidosis

    Directory of Open Access Journals (Sweden)

    Marcos Almeida Matos

    2013-04-01

    Full Text Available OBJETIVO: Avaliar o gasto energético na marcha em pacientes com mucopolissacaridose, utilizando uma metodologia simples e aplicável ao ambiente clínico. MÉTODOS: Realizou-se estudo transversal comparando-se o gasto energético da marcha de 19 pacientes portadores de mucopolissacaridose (Grupo MPS com 19 indivíduos assintomáticos da comunidade (Grupo Comparação. O gasto energético foi mensurado em Cal por um relógio da marca Polar (modelo FT7 durante uma caminhada de 50 metros. Foram também avaliados idade, peso, altura, IMC, frequência cardíaca inicial, frequência cardíaca final, e tempo de marcha. RESULTADOS: O Grupo MPS teve gasto energético na marcha de 2,84 Cal(±1,01, versus 1,42 Cal(±0,51, sendo 100% maior que o Grupo Comparação; também em relação ao Grupo Comparação, o Grupo MPS teve frequência cardíaca inicial 22% maior, frequência cardíaca final 13% e tempo da caminhada 25% maiores. CONCLUSÕES: O gasto energético na marcha de pacientes com mucopolissacaridose é duas vezes mais alto em comparação com indivíduos assintomáticos e a metodologia usada para avaliação mostrou-se alternativa eficiente para o ambiente clínico convencional. Nível de Evidencia III, Estudo Transversal Comparativo.OBJECTIVE: The aim of this study is to evaluate energy expenditure during gait in patients with mucopolysaccharidosis using a simple methodology applicable to the clinical setting. METHODS: A cross-sectional study was carried out comparing energy expenditure during gait in 19 patients with mucopolysaccharidosis (MPS Group with 19 control individuals from the community (Comparison Group. Energy expenditure was measured in calories using a Polar telemetric watch (model FT7 in a 50-meters walking. Variables such as age, weight, height, BMI, initial hart rate, final hart rate, and gait time were recorded. RESULTS: MPS Group showed an expenditure during gait of 2.84 Cal (± 1.01 versus 1.42 Cal (± 0.51, being 100% higher

  7. Incidence and natural history of mucopolysaccharidosis type III in France and comparison with United Kingdom and Greece.

    Science.gov (United States)

    Héron, Bénédicte; Mikaeloff, Yann; Froissart, Roseline; Caridade, Guillaume; Maire, Irène; Caillaud, Catherine; Levade, Thierry; Chabrol, Brigitte; Feillet, François; Ogier, Hélène; Valayannopoulos, Vassili; Michelakakis, Helen; Zafeiriou, Dimitrios; Lavery, Lucy; Wraith, Ed; Danos, Olivier; Heard, Jean-Michel; Tardieu, Marc

    2011-01-01

    Sanfilippo syndrome, or mucopolysaccharidosis type III (MPSIII) is a lysosomal storage disease with predominant neurological manifestations in affected children. It is considered heterogeneous with respect to prevalence, clinical presentation, biochemistry (four biochemical forms of the disease referred to as MPSIIIA, B, C, and D are known), and causative mutations. The perspective of therapeutic options emphasizes the need for better knowledge of MPSIII incidence and natural history. We performed parallel retrospective epidemiological studies of patients diagnosed with MSPIII in France (n = 128), UK (n = 126), and Greece (n = 20) from 1990 to 2006. Incidences ranged from 0.68 per 100,000 live-births in France to 1.21 per 100,000 live-births in UK. MPSIIIA, which predominates in France and UK, was absent in Greece, where most patients have MPSIIIB. The study confirmed the large allelic heterogeneity of MPSIIIA and MPSIIIB and detected several yet undescribed mutations. Analysis of clinical manifestations at diagnosis and over a 6-7 years follow-up indicated that almost all patients, whatever the disease subtype, expressed neurological manifestations before the age of 5 years, including language acquisition delay, cognitive delay, and/or abnormal behavior. In contrast to relatively homogeneous early onset manifestations, disease progression showed significant variation depending on subtype and age at diagnosis. Different severities of disease progressions and different allele distribution between France and UK suggested that mutations are not equally deleterious, although genotype-phenotype correlation could not be established. Notwithstanding the rapidity of further clinical deterioration, all MPSIII patients suffer early onset devastating neurological manifestations that deserve early treatment when available. Copyright © 2010 Wiley-Liss, Inc.

  8. Identification of age-dependent motor and neuropsychological behavioural abnormalities in a mouse model of Mucopolysaccharidosis Type II

    Science.gov (United States)

    Gleitz, Hélène F. E.; O’Leary, Claire; Holley, Rebecca J.

    2017-01-01

    Severe mucopolysaccharidosis type II (MPS II) is a progressive lysosomal storage disease caused by mutations in the IDS gene, leading to a deficiency in the iduronate-2-sulfatase enzyme that is involved in heparan sulphate and dermatan sulphate catabolism. In constitutive form, MPS II is a multi-system disease characterised by progressive neurocognitive decline, severe skeletal abnormalities and hepatosplenomegaly. Although enzyme replacement therapy has been approved for treatment of peripheral organs, no therapy effectively treats the cognitive symptoms of the disease and novel therapies are in development to remediate this. Therapeutic efficacy and subsequent validation can be assessed using a variety of outcome measures that are translatable to clinical practice, such as behavioural measures. We sought to consolidate current knowledge of the cognitive, skeletal and motor abnormalities present in the MPS II mouse model by performing time course behavioural examinations of working memory, anxiety, activity levels, sociability and coordination and balance, up to 8 months of age. Cognitive decline associated with alterations in spatial working memory is detectable at 8 months of age in MPS II mice using spontaneous alternation, together with an altered response to novel environments and anxiolytic behaviour in the open-field. Coordination and balance on the accelerating rotarod were also significantly worse at 8 months, and may be associated with skeletal changes seen in MPS II mice. We demonstrate that the progressive nature of MPS II disease is also seen in the mouse model, and that cognitive and motor differences are detectable at 8 months of age using spontaneous alternation, the accelerating rotarod and the open-field tests. This study establishes neurological, motor and skeletal measures for use in pre-clinical studies to develop therapeutic approaches in MPS II. PMID:28207863

  9. Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field test.

    Directory of Open Access Journals (Sweden)

    Alex Langford-Smith

    Full Text Available Reliable behavioural tests in animal models of neurodegenerative diseases allow us to study the natural history of disease and evaluate the efficacy of novel therapies. Mucopolysaccharidosis IIIA (MPS IIIA or Sanfilippo A, is a severe, neurodegenerative lysosomal storage disorder caused by a deficiency in the heparan sulphate catabolising enzyme, sulfamidase. Undegraded heparan sulphate accumulates, resulting in lysosomal enlargement and cellular dysfunction. Patients suffer a progressive loss of motor and cognitive function with severe behavioural manifestations and premature death. There is currently no treatment. A spontaneously occurring mouse model of the disease has been described, that has approximately 3% of normal enzyme activity levels. Behavioural phenotyping of the MPS IIIA mouse has been previously reported, but the results are conflicting and variable, even after full backcrossing to the C57BL/6 background. Therefore we have independently backcrossed the MPS IIIA model onto the C57BL/6J background and evaluated the behaviour of male and female MPS IIIA mice at 4, 6 and 8 months of age using the open field test, elevated plus maze, inverted screen and horizontal bar crossing at the same circadian time point. Using a 60 minute open field, we have demonstrated that female MPS IIIA mice are hyperactive, have a longer path length, display rapid exploratory behaviour and spend less time immobile than WT mice. Female MPS IIIA mice also display a reduced sense of danger and spend more time in the centre of the open field. There were no significant differences found between male WT and MPS IIIA mice and no differences in neuromuscular strength were seen with either sex. The altered natural history of behaviour that we observe in the MPS IIIA mouse will allow more accurate evaluation of novel therapeutics for MPS IIIA and potentially other neurodegenerative disorders.

  10. Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field test.

    Science.gov (United States)

    Langford-Smith, Alex; Langford-Smith, Kia J; Jones, Simon A; Wynn, Robert F; Wraith, J E; Wilkinson, Fiona L; Bigger, Brian W

    2011-01-01

    Reliable behavioural tests in animal models of neurodegenerative diseases allow us to study the natural history of disease and evaluate the efficacy of novel therapies. Mucopolysaccharidosis IIIA (MPS IIIA or Sanfilippo A), is a severe, neurodegenerative lysosomal storage disorder caused by a deficiency in the heparan sulphate catabolising enzyme, sulfamidase. Undegraded heparan sulphate accumulates, resulting in lysosomal enlargement and cellular dysfunction. Patients suffer a progressive loss of motor and cognitive function with severe behavioural manifestations and premature death. There is currently no treatment. A spontaneously occurring mouse model of the disease has been described, that has approximately 3% of normal enzyme activity levels. Behavioural phenotyping of the MPS IIIA mouse has been previously reported, but the results are conflicting and variable, even after full backcrossing to the C57BL/6 background. Therefore we have independently backcrossed the MPS IIIA model onto the C57BL/6J background and evaluated the behaviour of male and female MPS IIIA mice at 4, 6 and 8 months of age using the open field test, elevated plus maze, inverted screen and horizontal bar crossing at the same circadian time point. Using a 60 minute open field, we have demonstrated that female MPS IIIA mice are hyperactive, have a longer path length, display rapid exploratory behaviour and spend less time immobile than WT mice. Female MPS IIIA mice also display a reduced sense of danger and spend more time in the centre of the open field. There were no significant differences found between male WT and MPS IIIA mice and no differences in neuromuscular strength were seen with either sex. The altered natural history of behaviour that we observe in the MPS IIIA mouse will allow more accurate evaluation of novel therapeutics for MPS IIIA and potentially other neurodegenerative disorders.

  11. Hyperactive behaviour in the mouse model of mucopolysaccharidosis IIIB in the open field and home cage environments.

    Science.gov (United States)

    Langford-Smith, A; Malinowska, M; Langford-Smith, K J; Wegrzyn, G; Jones, S; Wynn, R; Wraith, J E; Wilkinson, F L; Bigger, B W

    2011-08-01

    Mucopolysaccharidosis IIIB (MPS IIIB) is a lysosomal storage disorder characterized by severe behavioural disturbances and progressive loss of cognitive and motor function. There is no effective treatment, but behavioural testing is a valuable tool to assess neurodegeneration and the effect of novel therapies in mouse models of disease. Several groups have evaluated behaviour in this model, but the data are inconsistent, often conflicting with patient natural history. We hypothesize that this discrepancy could be due to differences in open field habituation and home cage behaviour. Eight-month-old wild-type and MPS IIIB mice were tested in a 1-h open field test, performed 1.5 h after lights on, and a 24-h home cage behaviour test performed after 24 h of acclimatization. In the 1-h test, MPS IIIB mice were hyperactive, with increased rapid exploratory behaviour and reduced immobility time. No differences in anxiety were seen. Over the course of the test, differences became more pronounced with maximal effects at 1 h. The 24-hour home cage test was less reliable. There was evidence of increased hyperactivity in MPS IIIB mice, however, immobility was also increased, suggesting a level of inconsistency in this test. Performance of open field analysis within 1-2 h after lights on is probably critical to achieving maximal success as MPS IIIB mice have a peak in activity around this time. The open field test effectively identifies hyperactive behaviour in MPS IIIB mice and is a significant tool for evaluating effects of therapy on neurodegeneration. © 2011 The Authors. Genes, Brain and Behavior © 2011 Blackwell Publishing Ltd and International Behavioural and Neural Genetics Society.

  12. Mucopolysaccharidosis type VI on enzyme replacement therapy since infancy: Six years follow-up of four children

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    Dafne D.G. Horovitz

    2015-12-01

    Full Text Available Clinical and biochemical improvements are reported on Mucopolysaccharidosis type VI (MPS VI patients on Enzyme Replacement Therapy (ERT with rhASB (galsulfase, Naglazyme®, and preclinical and clinical studies have shown clinical benefits of early initiation. We report four unrelated MPS VI children who began ERT as infants (ages 5 days–10 months. The three older patients showed the first clinical signs of MPS VI at baseline, also presenting different degrees of dysostosis multiplex, and two had mild heart disease. The two oldest also had mild facial coarseness, one had hearing conduction deficit and sleep disorder and the other corneal clouding at baseline. After six years on ERT, all four patients have normal urinary GAG values. Although they all showed normal motor and mental development, brain and cervical spine MRI images available from two of the older patients showed abnormalities, while the youngest child continues having normal images. The four patients presented slower progression of bone and joint disease when compared to their affected older siblings. It should be noticed that only two patients in this sample are currently below the 3rd percentile for height: the youngest who has a constitutional factor associated and the eldest who already presented frank dysostosis at 10 months of age. These findings confirm previous studies that report that skeletal features of the disease cannot be completely prevented despite early ERT. Heart disease already present in two of the four infants at baseline got worse over time and appeared in another patient, but the youngest child on ERT introduction still has a normal echocardiogram at six years of age; he also is the only one without corneal clouding after six years follow-up. Our results also suggest that early ERT prevented storage in spleen and liver and may also have improved or prevented progression of facial dysmorphic features, corroborating similar findings seen in previous studies

  13. Mucopolysaccharidosis enzyme production by bone marrow and dental pulp derived human mesenchymal stem cells.

    Science.gov (United States)

    Jackson, Matilda; Derrick Roberts, Ainslie; Martin, Ellenore; Rout-Pitt, Nathan; Gronthos, Stan; Byers, Sharon

    2015-04-01

    Mucopolysaccharidoses (MPS) are inherited metabolic disorders that arise from a complete loss or a reduction in one of eleven specific lysosomal enzymes. MPS children display pathology in multiple cell types leading to tissue and organ failure and early death. Mesenchymal stem cells (MSCs) give rise to many of the cell types affected in MPS, including those that are refractory to current treatment protocols such as hematopoietic stem cell (HSC) based therapy. In this study we compared multiple MPS enzyme production by bone marrow derived (hBM) and dental pulp derived (hDP) MSCs to enzyme production by HSCs. hBM MSCs produce significantly higher levels of MPS I, II, IIIA, IVA, VI and VII enzyme than HSCs, while hDP MSCs produce significantly higher levels of MPS I, IIIA, IVA, VI and VII enzymes. Higher transfection efficiency was observed in MSCs (89%) compared to HSCs (23%) using a lentiviral vector. Over-expression of four different lysosomal enzymes resulted in up to 9303-fold and up to 5559-fold greater levels in MSC cell layer and media respectively. Stable, persistent transduction of MSCs and sustained over-expression of MPS VII enzyme was observed in vitro. Transduction of MSCs did not affect the ability of the cells to differentiate down osteogenic, adipogenic or chondrogenic lineages, but did partially delay differentiation down the non-mesodermal neurogenic lineage. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Evolução motora de paciente com mucopolissacaridose tipo 1 Motor outcome in a patient with mucopolysaccharidosis type 1

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    Cristina Iwabe

    2010-09-01

    Full Text Available OBJETIVO: Relatar os aspectos funcionais de uma criança com mucopolissacaridose tipo 1, descrevendo suas principais alterações após breve tratamento fisioterapêutico. DESCRIÇÃO DE CASO: Criança com 34 meses, portadora de mucopolissacaridose tipo 1 iniciou tratamento fisioterapêutico, duas vezes por semana, durante 50 minutos, sendo avaliada mensalmente por meio das atividades propostas pela Escala de Desenvolvimento Motor (EDM, que analisa a motricidade fina e global, o equilíbrio, o esquema corporal e a organização temporal e espacial. Na primeira avaliação, a criança apresentava-se com 34 meses de idade cronológica e 18 meses de idade motora geral, com melhor pontuação no item organização espacial, segundo a EDM. Após quatro meses, a idade motora geral era de 20 meses; porém, houve melhora da motricidade global. COMETÁRIOS: A análise das atividades funcionais do paciente relatado demonstrou atraso no desenvolvimento motor. A intervenção terapêutica possibilitou evolução favorável, principalmente na motricidade global, mesmo tratando-se de um portador de doença evolutiva.OBJECTIVE: To report the functional aspects of a child with mucopolysaccharidosis type 1 and to describe its main dysfunctions after a brief physiotherapy intervention. CASE DESCRIPTION: A 34 months old child with mucopolysaccharidosis type 1 started a physiotherapy treatment twice a week (50 minutes each session. Her motor skills were monthly evaluated by the Motor Development Scale (MDS, which analyzes fine and global motor skills, balance, body schema and temporal and spatial organization. In the first evaluation, the child had 34 months of chronological age and 18 months of global motor age, with better performance in spacial organization, according to MDS. After four months, the child had 20 months of general motor age; however, the global motor skills improved. Comments: The analysis of the patient's functional activities showed motor delay

  15. Klüver-Bucy syndrome associated with a recessive variant in HGSNAT in two siblings with Mucopolysaccharidosis type IIIC (Sanfilippo C).

    Science.gov (United States)

    Hu, Hao; Hübner, Christoph; Lukacs, Zoltan; Musante, Luciana; Gill, Esther; Wienker, Thomas F; Ropers, Hans-Hilger; Knierim, Ellen; Schuelke, Markus

    2017-02-01

    Klüver-Bucy syndrome (KBS) comprises a set of neurobehavioral symptoms with psychic blindness, hypersexuality, disinhibition, hyperorality, and hypermetamorphosis that were originally observed after bilateral lobectomy in Rhesus monkeys. We investigated two siblings with KBS from a consanguineous family by whole-exome sequencing and autozygosity mapping. We detected a homozygous variant in the heparan-α-glucosaminidase-N-acetyltransferase gene (HGSNAT; c.518G>A, p.(G173D), NCBI ClinVar RCV000239404.1), which segregated with the phenotype. Disease-causing variants in this gene are known to be associated with autosomal recessive Mucopolysaccharidosis type IIIC (MPSIIIC, Sanfilippo C). This lysosomal storage disease is due to deficiency of the acetyl-CoA:α-glucosaminidase-N-acetyltransferase, which was shown to be reduced in patient fibroblasts. Our report extends the phenotype associated with MPSIIIC. Besides MPSIIIA and MPSIIIB, due to variants in SGSH and NAGLU, this is the third subtype of Sanfilippo disease to be associated with KBS. MPSIII should be included in the differential diagnosis of young patients with KBS.

  16. Klüver–Bucy syndrome associated with a recessive variant in HGSNAT in two siblings with Mucopolysaccharidosis type IIIC (Sanfilippo C)

    Science.gov (United States)

    Hu, Hao; Hübner, Christoph; Lukacs, Zoltan; Musante, Luciana; Gill, Esther; Wienker, Thomas F; Ropers, Hans-Hilger; Knierim, Ellen; Schuelke, Markus

    2017-01-01

    Klüver–Bucy syndrome (KBS) comprises a set of neurobehavioral symptoms with psychic blindness, hypersexuality, disinhibition, hyperorality, and hypermetamorphosis that were originally observed after bilateral lobectomy in Rhesus monkeys. We investigated two siblings with KBS from a consanguineous family by whole-exome sequencing and autozygosity mapping. We detected a homozygous variant in the heparan-α-glucosaminidase-N-acetyltransferase gene (HGSNAT; c.518G>A, p.(G173D), NCBI ClinVar RCV000239404.1), which segregated with the phenotype. Disease-causing variants in this gene are known to be associated with autosomal recessive Mucopolysaccharidosis type IIIC (MPSIIIC, Sanfilippo C). This lysosomal storage disease is due to deficiency of the acetyl-CoA:α-glucosaminidase-N-acetyltransferase, which was shown to be reduced in patient fibroblasts. Our report extends the phenotype associated with MPSIIIC. Besides MPSIIIA and MPSIIIB, due to variants in SGSH and NAGLU, this is the third subtype of Sanfilippo disease to be associated with KBS. MPSIII should be included in the differential diagnosis of young patients with KBS. PMID:27827379

  17. Illness Perception and Clinical Treatment Experiences in Patients with M. Maroteaux-Lamy (Mucopolysaccharidosis Type VI) and a Turkish Migration Background in Germany

    Science.gov (United States)

    Dilger, Hansjörg; Leissner, Linn; Bosanska, Lenka; Lampe, Christina; Plöckinger, Ursula

    2013-01-01

    Introduction Mucopolysaccharidosis VI (MPS VI) is an inherited lysosomal storage disease caused by a mutation of the gene for arylsulfatase B (ASB). Of the thirty-one patients registered in Germany, almost fifty percent have a Turkish migration background. MPS VI is treated by enzyme replacement therapy (ERT), which is time-consuming and expensive. Methods This interdisciplinary study explored the illness perceptions and clinical treatment experiences among ten MPS VI patients with a Turkish migration background in two centers for metabolic diseases (Berlin and Mainz, Germany). The clinical treatment situation was observed and semi-structured interviews were conducted with patients and health care personnel, in addition to participatory observation in four patients' everyday environments in Berlin. The data from the interviews, patient records, and personal field notes were encoded, cross-related, and analyzed. Results Patients' acknowledgement of the disease and coping strategies are influenced predominantly by the perception of their individual health status and the handling of the disease within their family. Patients' willingness to cooperate with treatment strategies is further modified by their knowledge of the disease and the relationships with their health care providers. In this analysis, cultural factors turned out to be marginally relevant. Conclusion As with other chronic and debilitating diseases, effective treatment strategies have to reach beyond delivering medication. Health care providers need to strengthen the support for patients with a migration background. In this regard, they should respect the patients' cultural and social background and their personal perception of the disease and the therapy. Yet structural and social aspects (clinical setting, family and educational background) may be more crucial here than “cultural barriers.” PMID:23826140

  18. Reduction in Brain Heparan Sulfate with Systemic Administration of an IgG Trojan Horse-Sulfamidase Fusion Protein in the Mucopolysaccharidosis Type IIIA Mouse.

    Science.gov (United States)

    Boado, Ruben J; Lu, Jeff Zhiqiang; Hui, Eric Ka-Wai; Pardridge, William M

    2018-02-05

    Mucopolysaccharidosis Type IIIA (MPSIIIA), also known as Sanfilippo A syndrome, is an inherited neurodegenerative disease caused by mutations in the lysosomal enzyme, N-sulfoglucosamine sulfohydrolase (SGSH), also known as sulfamidase. Mutations in the SGSH enzyme, the only mammalian heparan N-sulfatase, cause accumulation of lysosomal inclusion bodies in brain cells comprising heparan sulfate (HS) glycosaminoglycans (GAGs). Treatment of MPSIIIA with intravenous recombinant SGSH is not possible because this large molecule does not cross the blood-brain barrier (BBB). BBB penetration by SGSH was enabled in the present study by re-engineering this enzyme as an IgG-SGSH fusion protein, where the IgG domain is a chimeric monoclonal antibody (mAb) against the mouse transferrin receptor (TfR), designated the cTfRMAb. The IgG domain of the fusion protein acts as a molecular Trojan horse to deliver the enzyme into brain via transport on the endogenous BBB TfR. The cTfRMAb-SGSH fusion protein bound to the mouse TfR with high affinity, ED 50 = 0.74 ± 0.07 nM, and retained high SGSH enzyme activity, 10 043 ± 1003 units/mg protein, which is comparable to recombinant human SGSH. Male and female MPSIIIA mice, null for the SGSH enzyme, were treated for 6 weeks with thrice-weekly intraperitoneal injections of vehicle, 5 mg/kg of the cTfRMAb alone, or 5 mg/kg of the cTfRMAb-SGSH fusion protein, starting at the age of 2 weeks, and were euthanized 1 week after the last injection. Brain and liver HS, as determined by liquid chromatography-mass spectrometry, were elevated 30-fold and 36-fold, respectively, in the MPSIIIA mouse. Treatment of the mice with the cTfRMAb-SGSH fusion protein caused a 70% and 85% reduction in brain and liver HS, respectively. The reduction in brain HS was associated with a 28% increase in latency on the rotarod test of motor activity in male mice. The mice exhibited no injection related reactions, and only a low titer end of study antidrug antibody

  19. I I I I I I

    African Journals Online (AJOL)

    Our findings are in keeping with other reports that VIP levels rise in the portal venous blood in mesenteric ischaemia.6 The mean NT levels in the peripheral venous samp!es in group I showed a decreasing trend, which was also see~ m the por::~ .... portal vein in such a fashion that ;the portal venous blood flow was not ...

  20. Audiological findings in children with mucopolysaccharidoses type i-iv.

    Science.gov (United States)

    Vargas-Gamarra, María F; de Paula-Vernetta, Carlos; Vitoria Miñana, Isidro; Ibañez-Alcañiz, Isabel; Cavallé-Garrido, Laura; Alamar-Velazquez, Agustín

    The aim of our study is to reflect hearing impairment of 23children diagnosed with mucopolysaccharidosis (MPS) typeI, II, III and IV. Retrospective study of the clinical, audiological and treatment (medical vs surgical) findings of 23children diagnosed with MPS typeI, II, III or IV followed at a Tertiary Referral Hospital between 1997 and 2015. Six cases of MPSI, 8 of MPSII, 4 of MPSIII and 5 of MPSIV were reviewed. 71.2% of patients had secretory otitis media (SOM) and 54% of patients had some type of hearing loss (HL). The behaviour of hearing loss was variable in each of the subgroups of MPS, finding greater involvement and variability in typesI and II. Children with MPS have a high risk of hearing loss. A significant percentage of transmissive HL progressing to mixed or sensorineural HL was observed. This was more common in typesI and II. Periodic follow up of these patients is mandatory because of hearing impairment and consequences for their development and quality of life. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  1. Consequências da judicialização das políticas de saúde: custos de medicamentos para as mucopolissacaridoses Consequences of the judicialization of health policies: the cost of medicines for mucopolysaccharidosis

    Directory of Open Access Journals (Sweden)

    Debora Diniz

    2012-03-01

    Full Text Available O estudo analisa os gastos da judicialização de medicamentos para a mucopolissacaridose (MPS, uma doença rara, de alto custo, fora da política de assistência farmacêutica e com benefício clínico. O levantamento de dados foi realizado nos arquivos de 196 dossiês que determinou que o Ministério da Saúde fornecesse medicamentos no período entre 2006 e 2010, e nos registros administrativos e contábeis do Ministério da Saúde. A análise identifica sujeição do governo brasileiro a monopólios de distribuição de medicamentos e, consequentemente, perda de sua capacidade de administrar compras. Também identifica que a imposição da aquisição imediata e individualizada impede a obtenção de economias de escala com a compra planejada de maiores quantidades de medicamento, e impõe dificuldades logísticas para o controle das quantidades consumidas e estocadas. Conclui-se que a judicialização decorre da ausência de uma política clara do sistema de saúde para doenças raras em geral, e tem como consequência gastos acima do necessário para o tratamento.This study analyzes expenditures backed by court rulings to ensure the public provision of medicines for treatment of mucopolysaccharidosis (MPS, a rare disease that requires high-cost drugs not covered by the Brazilian government's policy for pharmaceutical care and which have disputed clinical efficacy. The methodology included a review of files from 196 court rulings ordering the Brazilian Ministry of Health to provide the medicines, in addition to Ministry of Health administrative records. According to the analysis, the "judicialization" of the health system subjected the Brazilian government to a monopoly in the distribution of medicines and consequently the loss of its capacity to manage drug purchases. The study also indicates that the imposition of immediate, individualized purchases prevents obtaining economies of scale with planned procurement of larger amounts of the

  2. Substrate Deprivation Therapy to Reduce Glycosaminoglycan Synthesis Improves Aspects of Neurological and Skeletal Pathology in MPS I Mice

    Directory of Open Access Journals (Sweden)

    Ainslie L. K. Derrick-Roberts

    2017-02-01

    Full Text Available Mucopolysaccharidosis type I (MPS I is the most common form of the MPS group of genetic diseases. MPS I results from a deficiency in the lysosomal enzyme α-l-iduronidase, leading to accumulation of undegraded heparan and dermatan sulphate glycosaminoglycan (GAG chains in patient cells. MPS children suffer from multiple organ failure and die in their teens to early twenties. In particular, MPS I children also suffer from profound mental retardation and skeletal disease that restricts growth and movement. Neither brain nor skeletal disease is adequately treated by current therapy approaches. To overcome these barriers to effective therapy we have developed and tested a treatment called substrate deprivation therapy (SDT. MPS I knockout mice were treated with weekly intravenous injections of 1 mg/kg rhodamine B for six months to assess the efficacy of SDT. Mice were assessed using biochemistry, micro-CT and a battery of behaviour tests to determine the outcome of treatment. A reduction in female bodyweight gain was observed with the treatment as well as a decrease in lung GAG. Behavioural studies showed slight improvements in inverted grid and significant improvements in learning ability for female MPS I mice treated with rhodamine B. Skeletal disease also improved with a reduction in bone mineral volume observed. Overall, rhodamine B is safe to administer to MPS I knockout mice where it had an effect on improving aspects of neurological and skeletal disease symptoms and may therefore provide a potential therapy or adjunct therapy for MPS I patients.

  3. Neonatal tolerance induction enables accurate evaluation of gene therapy for MPS I in a canine model.

    Science.gov (United States)

    Hinderer, Christian; Bell, Peter; Louboutin, Jean-Pierre; Katz, Nathan; Zhu, Yanqing; Lin, Gloria; Choa, Ruth; Bagel, Jessica; O'Donnell, Patricia; Fitzgerald, Caitlin A; Langan, Therese; Wang, Ping; Casal, Margret L; Haskins, Mark E; Wilson, James M

    2016-09-01

    High fidelity animal models of human disease are essential for preclinical evaluation of novel gene and protein therapeutics. However, these studies can be complicated by exaggerated immune responses against the human transgene. Here we demonstrate that dogs with a genetic deficiency of the enzyme α-l-iduronidase (IDUA), a model of the lysosomal storage disease mucopolysaccharidosis type I (MPS I), can be rendered immunologically tolerant to human IDUA through neonatal exposure to the enzyme. Using MPS I dogs tolerized to human IDUA as neonates, we evaluated intrathecal delivery of an adeno-associated virus serotype 9 vector expressing human IDUA as a therapy for the central nervous system manifestations of MPS I. These studies established the efficacy of the human vector in the canine model, and allowed for estimation of the minimum effective dose, providing key information for the design of first-in-human trials. This approach can facilitate evaluation of human therapeutics in relevant animal models, and may also have clinical applications for the prevention of immune responses to gene and protein replacement therapies. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. A tese da judicialização da saúde pelas elites: os medicamentos para mucopolissacaridose The thesis of judicialization of health care by the elites: medication for mucopolysaccharidosis

    Directory of Open Access Journals (Sweden)

    Ida Vanessa Doederlein Schwartz

    2013-04-01

    Full Text Available O artigo avalia a hipótese de se a judicia­lização de medicamentos para o tratamento das mucopolissacaridoses no Brasil seria uma ação das elites econômicas. Debatem-se estudos prévios que defendem a tese da judicialização pelas elites em outros medicamentos. Discute-se, a metodologia desses estudos e as inferências dela derivadas e o respaldo empírico dessa tese no caso de um dos medicamentos judicializados de mais alto custo para o SUS. Foram analisados os 196 processos julgados entre fevereiro de 2006 e dezembro de 2010 que determinam a provisão gratuita dos medicamentos para mucopolissacaridoses pelo Ministério da Saúde. Há evidências de que os custos advocatícios sejam financiados por entidades interessadas nos resultados da judicialização, como as empresas distribuidoras ou indústrias farmacêuticas, de que pode haver migração dos pacientes para diagnóstico e tratamentos em centros universitários de referência para a inovação médica no país, e de que a opção por serviços públicos se dá por sua capacidade técnica e científica supe­rior à de outras instituições. Logo, a advocacia privada, indicadores de exclusão social do local de residência dos pacientes e uso de serviços públicos não são informações de classe que corroborem ou refutem a tese da judicialização pelas elites.This paper evaluates the hypothesis that the judicialization of medicine for mucopolysaccharidosis in Brazil is an action promoted by economic elites. Previous studies upholding the thesis of judicialization by elites in the case of other types of medication that are more costly for the Unified Health Service are discussed. An analysis of all 196 processes containing information about judicial processes brought to court between February 2006 and December 2010 that ended by determining that the State should provide such medication free of charge to patients was conducted. There is evidence that attorneys' fees were covered

  5. Assessment of dysmyelination with RAFFn MRI: application to murine MPS I.

    Directory of Open Access Journals (Sweden)

    David Satzer

    Full Text Available Type I mucopolysaccharidosis (MPS I is an autosomal recessive lysosomal storage disorder with neurological features. Humans and laboratory animals with MPS I exhibit various white matter abnormalities involving the corpus callosum and other regions. In this study, we first validated a novel MRI technique, entitled Relaxation Along a Fictitious Field in the rotating frame of rank n (RAFFn, as a measure of myelination and dysmyelination in mice. We then examined differences between MPS I mice and heterozygotes using RAFF5 and histology. RAFF5 (i.e., RAFFn with n = 5 relaxation time constants were highly correlated with histological myelin density (R2 = 0.68, P<0.001, and RAFF5 clearly distinguished between the hypomyelinated and dysmyelinated shiverer mouse and the wild-type mouse. Bloch-McConnell theoretical analysis revealed slower exchange correlation times and smaller exchange-induced relaxation rate constants for RAFF4 and RAFF5 compared to RAFF1-3, T1ρ, and T2ρ. These data suggest that RAFF5 may assess methylene protons in myelin lipids and proteins, though other mechanisms (e.g. detection of myelin-bound water may also explain the sensitivity of RAFF5 to myelin. In MPS I mice, mean RAFF5 relaxation time constants were significantly larger for the striatum (P = 0.004 and internal capsule (P = 0.039, and marginally larger for the fornix (P = 0.15. Histological assessment revealed no differences between MPS I mice and heterozygotes in myelin density or corpus callosum thickness. Taken together, these findings support subtle dysmyelination in the brains of mice with MPS I. Dysmyelination may result from myelin lipid abnormalities caused by the absence of α-L-iduronidase. Our findings may help to explain locomotor and cognitive deficits seen in mice with MPS I.

  6. Clinical Presentation of Mucopolysaccharidosis Type II (Hunter's ...

    African Journals Online (AJOL)

    This report is of a 7-year-old boy who presented to the children outpatient through a referral with a history of inability to grasp objects, inability to express self, and coarse skin, which started 5 years ago. On examination, he was short statured, with a big head, protruding abdomen, coarse skin, swollen wrist joints, and ...

  7. Genetics Home Reference: mucopolysaccharidosis type II

    Science.gov (United States)

    ... but they typically live into adulthood and their intelligence is not affected. Heart disease and airway obstruction are major causes of death in people with both types of MPS II. Related Information What does it ...

  8. Genetics Home Reference: mucopolysaccharidosis type III

    Science.gov (United States)

    ... anxious, or aggressive, and some display features of autism spectrum disorder , which is a condition characterized by difficulty with social interactions and communication. Sleep disturbances are also very common in children ...

  9. Genetics Home Reference: mucopolysaccharidosis type VI

    Science.gov (United States)

    ... accumulation of GAGs within cells, specifically inside the lysosomes . Lysosomes are compartments in the cell that digest and ... that cause molecules to build up inside the lysosomes are called lysosomal storage disorders. The accumulation of ...

  10. Genetics Home Reference: mucopolysaccharidosis type IV

    Science.gov (United States)

    ... enzymes, GAGs accumulate within cells, specifically inside the lysosomes . Lysosomes are compartments in the cell that break down ... that cause molecules to build up inside the lysosomes are called lysosomal storage disorders. In MPS IV, ...

  11. Genetics Home Reference: mucopolysaccharidosis type VII

    Science.gov (United States)

    ... accumulation of GAGs within cells, specifically inside the lysosomes . Lysosomes are compartments in the cell that digest and ... that cause molecules to build up inside the lysosomes are called lysosomal storage disorders. The accumulation of ...

  12. Profile of Egyptian Patients with Mucopolysaccharidosis | Shawky ...

    African Journals Online (AJOL)

    Background: Mucopolysaccharidoses (MPS) are chronic progressive lysosomal disorders (Six distinct types) which are inherited as autosomal recessive except MPS II which is inherited as X-linked recessive disorder. Patients and Methods: This study is designed to investigate a group of Egyptian patients with MPS ...

  13. Clinical Presentation of Mucopolysaccharidosis Type II (Hunter's ...

    African Journals Online (AJOL)

    hanumantp

    MPS is a group of metabolic disorders caused by absence or malfunctioning of the lysosomal enzymes needed to break down molecules called glycosaminoglycans (GAGs).[1]. Children with MPs either do not produce enough of one of the two enzymes required to break down the sugar chains into proteins and simpler ...

  14. Residual glycosaminoglycan accumulation in mitral and aortic valves of a patient with attenuated MPS I (Scheie syndrome after 6 years of enzyme replacement therapy: Implications for early diagnosis and therapy

    Directory of Open Access Journals (Sweden)

    Yohei Sato

    2015-12-01

    Full Text Available Mucopolysaccharidosis (MPS is an inherited metabolic disease caused by deficiency of the enzymes needed for glycosaminoglycan (GAG degradation. MPS type I is caused by the deficiency of the lysosomal enzyme alpha-l-iduronidase and is classified into Hurler syndrome, Scheie syndrome, and Hurler–Scheie syndrome based on disease severity and onset. Cardiac complications such as left ventricular hypertrophy, cardiac valve disease, and coronary artery disease are often observed in MPS type I. Enzyme replacement therapy (ERT has been available for MPS type I, but the efficacy of this treatment for cardiac valve disease is unknown. We report on a 56-year-old female patient with attenuated MPS I (Scheie syndrome who developed aortic and mitral stenosis and coronary artery narrowing. The cardiac valve disease progressed despite ERT and she finally underwent double valve replacement and coronary artery bypass grafting. The pathology of the cardiac valves revealed GAG accumulation and lysosomal enlargement in both the mitral and aortic valves. Zebra body formation was also confirmed using electron microscopy. Our results suggest that ERT had limited efficacy in previously established cardiac valve disease. Early diagnosis and initiation of ERT is crucial to avoid further cardiac complications in MPS type I.

  15. <i>Laserpitium> gr. <i>nestleri> (Umbelliferae

    Directory of Open Access Journals (Sweden)

    Montserrat, Pedro

    2003-12-01

    Full Text Available For the revision of the genus <i>Laserpitium> for Flora lberica I have revised numerous materials of many herbaria. In this paper I present! the result of a morphological and taxonomic study of <i>L. nestlerii> and <i>L. eliasiii>, supported with numerous iIIustrations only partially included here. <i>L. nestlerii> subsp. <i>nestleri> is distributed from Central France (Central Massif to the SE of the lberian Peninsula. Two new subspecies allow to relate the morphological variability observed with their geographical distribution. <i>L. nestlerii> subsp. <i>flabellatum> is found in the Pyrenees and Ihe Cantabrian mountains, and <i>L. nestlerii> subsp. <i>lainzii> is a remarkable endemism located in the mountains of the N of Leon. <i>L. eliasiii> also presents a great morphological variability. Their distribution area is overlapped partially with that of <i>L. nestlerii> subsp. <i>flabellatum>. <i>L. eliasiii> subsp. <i>ordunae> include forms located in the Basque country while the most western populations, from W Galicia and N of Portugal, belong to <i>L. eliasiii> subsp. <i>thalictrifolium>. I propose different new varieties to highlight some remarkable local forms. The study of mericarp section. the distribution of estomate and the morphology of hairs and basal leaves are very useful for delimitate these taxa.

    Con motivo de la revisión del género <i>Laserpitium> para Flora Ibérica he revisado mucho material de distintos herbarios. En este trabajo presento el resultado de un estudio morfológico y taxonómico de las especies <i>L. nestlerii> y <i>L. eliasiii
    >, apoyado en numerosas ilustraciones. de las cuales sólo una pequeña parte se incluyen en este artículo. <i>L. nestlerii> subsp. <i>nestleri> se distribuye desde el Centro de Francia (Massif Central hasta el SE de la Península Ibérica. Dos nuevas subespecies permiten relacionar la variabilidad morfológica observada con su

  16. 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.

  17. Beboerdeltagelse i saneringsprocessen i Ringsted

    DEFF Research Database (Denmark)

    Larsen, V

    I 3.delrapport redegøres for en interviewundersøgelse der belyser beboernes holdninger til saneringsplan og lokalplan for deres boligområde, til de gennemførte offentlighedsaktiviteter og til det at deltage i en planlægningsproces. Meddelelsen konkluderer i forslag til forbedringer i offentlighed...

  18. <i>Editorial>

    Directory of Open Access Journals (Sweden)

    Irene Machado

    2008-11-01

    ção Regina nos conta na seção de Galáxia. Na seção de resenhas, este número traz ainda análise de obras imprescindí­veis: Da Imperfeição, de J. Greimas, por Lúcia Teixeira; O instinto da linguagem, de Steve Pinker, por Mirna Feitoza; Interlab. Labirintos do pensamento contemporâneo, organizado por Lucia Leão, por Geane Alzamora; Transformação e Realidade: mundos convergentes e divergentes, organizado por Dulcimira Capisani, por Eluí­za Ghizzi; Tekstura: Russian essays on visual culture, organizado por Alla Efimova e Lev Manovich, por Jeová Rocha; Sinestesia, arte e tecnologia: fundamentos da cromossonia, de Sérgio Roclaw Basbaum, por Eufrásio Prates; Ática, Cidadania e Imprensa, organizado por Raquel Paiva, por Djalma Benette. Duas notí­cias: uma sobre a reunião da COMPÔS, Associação dos Programas de Pós-Graduação em Comunicação, e outra sobre a I Conferência de Semiótica, promovido pela Associação Brasiliense de Comunicação e Semiótica de Brasí­lia. Com essa gama de temas e problemas; teorias e abordagens, Galáxia preserva a seu leitor o direito de avaliar a performance por meio da qual tem enfrentado os debates de temas de sua área de competência e dentro de sua proposta editorial. Irene Machado Editora Cientí­fica

  19. Early treatment with laronidase improves clinical outcomes in patients with attenuated MPS I: a retrospective case series analysis of nine sibships.

    Science.gov (United States)

    Al-Sannaa, Nouriya A; Bay, Luisa; Barbouth, Deborah S; Benhayoun, Youssef; Goizet, Cyril; Guelbert, Norberto; Jones, Simon A; Kyosen, Sandra Obikawa; Martins, Ana Maria; Phornphutkul, Chanika; Reig, Celia; Pleat, Rebecca; Fallet, Shari; Ivanovska Holder, Iva

    2015-10-07

    Enzyme replacement therapy (ERT) with laronidase, (recombinant human α-L-iduronidase; Aldurazyme) is the primary treatment option for patients with attenuated mucopolysaccharidosis type I (MPS I). This study examined the effect of early ERT on clinical manifestations. This multinational, retrospective case series abstracted data from records of 20 patients with Hurler-Scheie syndrome within nine sibships that included older siblings treated with laronidase after the development of significant clinical symptoms, and younger siblings treated before significant symptomatology. Median age at diagnosis was 5.6 and 0.5 years for older and younger siblings, respectively. Median age at ERT initiation was 7.9 and 1.9 years for older and younger siblings, respectively. Improvement or stabilization of somatic signs and symptoms was more notable in younger siblings. Organomegaly present at onset of ERT improved in the majority of both older and younger siblings. Analysis of physician-rated symptom severity demonstrated that cardiac, musculoskeletal, and cognitive symptoms, when absent or mild in younger siblings at ERT initiation, generally did not develop or progress. The majority of older siblings had height/length Z-scores greater than two standard deviations below the mean (less than -2) at both time points. In general, Z-scores for younger siblings were closer to the sex- and age-matched means at follow-up. These findings suggest early initiation of laronidase, prior to the onset of symptoms in patients with attenuated MPS I, can slow or prevent the development of severe clinical manifestations.

  20. Happy i

    International Nuclear Information System (INIS)

    Lee, Seung Hui

    2008-10-01

    This book introduces the center i in which, people experiences energy with their whole body. It also gives descriptions of solar energy as source of life, fossil energy in various shape, nuclear energy made by nuclear fission, history of using energy, future energy like wind energy, ocean energy, bio energy, geothermal energy, hydrogen energy, new regeneration energy, introduction of experiences in center i.

  1. ALCOHOL I

    African Journals Online (AJOL)

    Despite the increase in alcohol marketing activities by the transnational alcohol corporations in Nigeria .... were recorded with a digital device with ..... era (i.e., before alcohol industry was es- tablished in ..... university student drinking: A na-.

  2. Metaphysics I

    OpenAIRE

    Lebech, Mette

    2003-01-01

    Metaphysics I is a course currently taught in the Faculty of Philosophy, NUIM. It attempts to answer three questions: 'What is metaphysics?', 'What happened as metaphysics was Christianised?' and 'What happenerd as metaphysics was Modernized?', while discussing texts of Aristotle, Thomas Aquinas, Edmund Husserl and Edith Stein.

  3. Panskoye I

    DEFF Research Database (Denmark)

    Stolba, Vladimir

    2017-01-01

    Panskoye I is one of the most prominent and best-studied settlements in the rural territory of Chersonesus on the Tarkhankut Peninsula (north-western Crimea). Founded in the late 5th century BCE as a fortified outpost (tetrapyrgia) protecting the south-eastern frontiers of Olbian territory, aroun...

  4. The neurobehavioral phenotype in mucopolysaccharidosis Type IIIB: An exploratory study

    Directory of Open Access Journals (Sweden)

    E. Shapiro

    2016-03-01

    Conclusions: On most behavioral measures, MPS IIIB patients did not differ substantially from MPS IIIA patients over age six, demonstrating autistic features and a Klüver Bucy-like syndrome including lack of fear and poor attention. Delay in onset of behavioral symptoms was associated with later diagnosis in two patients. Lack of fear, poor attention, and autistic-like symptomatology are as characteristic of MPS IIIB as they are of MPS IIIA. A possible difference is that the some behavioral abnormalities develop more quickly in MPS IIIB. If this is so, these patients may become at risk for harm and present a challenge for parenting even earlier than do those with MPS IIIA. In future clinical trials of new treatments, especially with respect to quality of life and patient management, improvement of these behaviors will be an essential goal. Because very young patients were not studied, prospective natural history documentation of the early development of abnormal behaviors in MPS IIIB is needed.

  5. Cytotaxonomy of North African species of <i>Delphinium> L. sect. <i>Delphinium> (<i>Ranunculaceae>

    Directory of Open Access Journals (Sweden)

    Blanché, César

    1990-05-01

    Full Text Available Both chromosome numbers and karyotype structure from 15 wild North-African populations of <i>Delphinium> L. sect. <i>Delphinium> were studied. <i>Delphinium balansaei> Boiss. & Reuter, with a more symmetrical karyotype, appears as a possible perennial ancestor of' the remaining annuals with highly asymmetrical and shorter karyotype. A taxonomical reorganization of this section is proposed: two new series (ser. <i>Macropetala> ser. nova and ser. <i>Balansae> ser. nova. and two new nomenclatural combinations (<i>D. nanumi> subsp. <i>alboliliaceum> and <i>D. nanumi> subsp. <i>elongatum> are proposed.

    S'han estudiat els nombres cromosòmics i l'estructura deis cariotipus de 15 poblacions nordafricanes de <i>Delphinium> L. sect. <i>Delphinium. Delphinium balansaei> Boiss. & Reuter, amb cariotipus mes simètric, apareix com a possible ancestre perenne de la resta d’espècies anuals amb cariotipus més asimètric i més curt. Es proposa una reorganització taxonòmica de la secció, d'on es descriu en dues series noves (ser. <i>Macropetala> ser. nova i ser. <i>Balansae> ser. nova. i es proposen dues combinacions noves (<i>D. nanumi> subsp. <i>alboliliaceum> and <i>D. nanumi> subsp. <i>elongatum>.

  6. TLF i .

    Directory of Open Access Journals (Sweden)

    Jacques Lévy

    2004-01-01

    Full Text Available Ce site présente la version numérisée du Trésor de la langue française , un ouvrage réalisé par le C nrs et édité sur papier, en 23 000 pages et seize volumes. D'où le titre du site : Tlf i , i pour « informatisé ». Ce site est géré par l'unité de recherche Analyse et traitement informatique de la langue française (Atilf, Cnrs /Nancy-2. Son accès est libre. L'entreprise Tlf reprenait le titre donné par Jean Nicot en 1606 ( Thresor de la langue ...

  7. Spanish I

    CERN Document Server

    Rodriguez, Jill

    2001-01-01

    CliffsQuickReview course guides cover the essentials of your toughest classes. Get a firm grip on core concepts and key material, and test your newfound knowledge with review questions. CliffsQuickReview Spanish I is meant to provide all the foundations of basic Spanish pronunciation, spelling, and sentence construction. Spanish grammar is systematically explained in its most simplistic way, so there's no need for any prerequisite before beginning this ""review"" of the equivalent of two years of high school Spanish. As you work your way through this review, you'll be ready to tackle such conc

  8. Multidisciplinary Team Approach Is Key for Managing Pregnancy and Delivery in Patient with Rare, Complex MPS I.

    Science.gov (United States)

    Troko, J; Poonawala, Y; Geberhiwot, T; Martin, B

    2016-01-01

    A 23-year-old primiparous lady (Ms S) was referred to preconception clinic with known Hurler-Scheie syndrome (mucopolysaccharidosis 1). Ms S had been under the care of the adult inherited metabolic disorder physicians prior to becoming pregnant. She and her partner received prenatal counselling and following spontaneous conception was closely managed by a multidisciplinary team involving foetomaternal obstetricians, anaesthetists, cardiologists, geneticists and endocrinologists in two tertiary referral hospitals throughout her pregnancy. She went on to deliver a live male child at 37/40 by elective caesarean section. As far as we are aware, this is the first case report of a term pregnancy in a woman with moderate to severe mucopolysaccharidosis 1 (MPS 1).

  9. Luvica I

    Directory of Open Access Journals (Sweden)

    Bojan Čop

    1956-12-01

    Full Text Available Dans son compte-rendu de Otten, Z. gramm. und lex. Bestimmung des Luv., M. Laroche a éclairé d'une manière incontestable le sens de ce mot: BiOr. XI, 1954, p. 124, il montre que ulantiš sert d'opposé à huiiduuališ de la ligne précédente; pour celui-ci, Otten, op. cit., p. 86, a démontré le sens de «vivant»; ulantiš signifie par là «mort». Le mot est, en tant que thème en -i-, membre d'une déclinaison louvite, qui a vastement dépassé les limites dessinées par l'indo-européen commun: le -i- sert d'élargissement pour toute sorte d e thèmes consonantiques; ainsi apparant- «futur» devient en louvite apparanti-, tijamm- «terre» passe à tiiammi-, v. ci-dessous, etc. Notre mot sera donc élargi de *ulant-; et celui-ci a l'air d'un participe ou au moins d'un adjectif du type connu du hittite: hitt. akkant- «mort», part, du présent de akk- « mourir» ; maklant- «maigre», adj. en -ant-, de même irmalant- «malade», etc. Si ulant- est participe, on le déduira de ul-, thème verbal au sens de «périr», «mourir», donc au sens intrans, comme hitt. akkant-, ou bien on y verra un participe en -nt- au sens passif, dont la base verbale sera transitive, au sens de «faire périr, anéantir, tuer»; or il existe une racine indo-européenne qui montre les deux sens, le transitif et l' intransitif, réunis dans le même système: c'est *uel-(ə- «tuer» et «périr», à savoir dans hittite цаlh- «frapper, tuer», v. pruss. ūlint «lutter», en face de lait. vallessit «perierit», v. norr. valr «les cadavres sur le champ de bataille», tolkh. A αäl- «mourir», etc.;33 voir Pedersen, Tocharisch, p. 189.

  10. Calculus I

    CERN Document Server

    Knight, Brian

    1975-01-01

    Each chapter in this book deals with a single mathematical topic, which ideally should form the basis of a single lecture. The chapter has been designed as a mixture of the following ingredients: -(i) Illustrative examples and notes for the student's pre-lecture reading. (ii) Class discussion exercises for study in a lecture or seminar. (iii) Graded problems for assignment work. Contents 1 Sets, functions page 11 2 Limits and continuity 17 3 The exponential and related functions 25 4 Inverse functions 30 5 Differentiation 35 6 Differentiation of implicit functions 44 7 Maxima and minima 50 8 Curve sketching 54 9 Expansion in series 61 10 Newton's method 67 11 Area and integration 72 12 Standard integrals 80 13 Applications of the fundamental theorem 87 14 Substitution in integrals 94 15 Use of partial fractions 100 16 Integration by parts 106 Answers to problems 110 Index 116 1 Sets, Functions A set is a collection of distinct objects. The objects be­ longing to a set are the elements (or members) of the set...

  11. Digital dannelse i literacypraksis i dagtilbud

    DEFF Research Database (Denmark)

    Frydendahl, Jette Aabo

    2016-01-01

    De digitale medier har i det sidste årti fået en omfattende udbredelse og anvendelse i både skole og dagtilbud. Mange dagtilbud har i dag en pæn maskinpark af iPads, tablets, digitale tavler og forskellige spilkonsoller. Når man skal arbejde mediepædagogisk i dagtilbud, er det nødvendigt at forho......De digitale medier har i det sidste årti fået en omfattende udbredelse og anvendelse i både skole og dagtilbud. Mange dagtilbud har i dag en pæn maskinpark af iPads, tablets, digitale tavler og forskellige spilkonsoller. Når man skal arbejde mediepædagogisk i dagtilbud, er det nødvendigt...

  12. Muslim i Danmark - muslim i verden

    DEFF Research Database (Denmark)

    Schmidt, Garbi

    muslimske ungdomsmiljø i det hele taget et homogent miljø, eller viser det os islam i Danmark som et flerstemmigt fænomen, præget af indre debat om, hvilken rolle islam skal spille nationalt og globalt? Er islam i disse sammenhænge et spirituelt budskab eller en politisk ideologi; en individuel vejledning...

  13. Kina i Zambia: Indsigt i kinesiske investeringer i Zambias kobberminer

    OpenAIRE

    Tewolde, Eden; Boateng, Amanda

    2016-01-01

    Projektet tager udgangspunkt i Kinas investeringer i Zambias Kobberminer og fokusere på de to landes relationer med hinanden, samt de konsekvenser der forekommer af Kinas privatiseringer af Zambias kobbersektor. Projektet har en økonomisk og social tilgang, hvor Kinas FDI i Zambia, bliver analyseret for at se på hvor stor en økonomisk vækst der skabes i Zambia på vejne af udlandske investeringer. Derudover er der også blevet gjort brug af ressourceforbandelsens teori, til at se på de sociale,...

  14. Verden i Norden - Norden i Verden

    DEFF Research Database (Denmark)

    Duelund, Peter

    Det nordiske kultursamarbejde har siden kulturaftalen mellem de nordiske lande i 1971 udvikling sig i flere retninger. Artiklen er udarbejdet på opfordring af Nordisk Ministerråd, som et afsæt for at beskrive, analysere og vurdere det nordiske kultursamarbejde historiske udvikling og fremtidige...... udfordringer i en globaliseret verden. Hvor bør de nordiske landes kulturpolitik og det nordiske kultursamarbejde tage fat ved indgangen til det 21. århundrede, hvis Norden skal sætte sig spor i Europa og den øvrige verden med udgangspunkt i den velfærdsbaserede kulturpolitik, der er oparbejdet efter 2...

  15. Mod i ledelse

    DEFF Research Database (Denmark)

    Mellon, Karsten

    2016-01-01

    Mod i ledelse er en efterspurgt vare i offentligt regi som modsvar på stigende kompleksitet og pres. Men hvad er ’mod i ledelse’ – og er du selv en modig leder?......Mod i ledelse er en efterspurgt vare i offentligt regi som modsvar på stigende kompleksitet og pres. Men hvad er ’mod i ledelse’ – og er du selv en modig leder?...

  16. I-1 General; I-1 Opsti deo

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1960-07-01

    General part of the regulations and instructions for operation of the RA reactor includes fundamental data about the reactor properties, biological shields, purpose of reactor operation, organizational scheme, rights and responsibilities of the head of laboratory, leaders of the working teams and all the staff of the laboratory for RA reactor exploitation. Opsti deo knjige propisa i uputstva za rad reaktora sadrzi osnovne podatke o reaktoru, biloskoj zastiti, nameni reaktora, prava i duznosti nacelnika, sefova smena i osoblja laboratorije za eksploataciju reaktora RA.

  17. Atlantvolden i Danmark

    DEFF Research Database (Denmark)

    Christensen, Claus Bundgård

    2014-01-01

    Anmeldelse af Lulu Anne Hansen & Martin Rheinheimer (red.): Bunkers. Atlantvoldens perspektiver i Danmark.......Anmeldelse af Lulu Anne Hansen & Martin Rheinheimer (red.): Bunkers. Atlantvoldens perspektiver i Danmark....

  18. Projekt i innovation

    OpenAIRE

    Rasmussen, Dorte Sams Marstrand

    2017-01-01

    AbstraktProjektet ”Projekt i innovation”, tager afsæt i et HR-ledelsesperspektiv i organisationen Google, hvor det undersøges, hvordan innovation udspiller sig i praksis, hvilket sker gennem i en komparativ analyse, med en ansat medarbejder i organisationen. Projektet har til formål at undersøge, om der i organisationen eksisterer særlig tilgange til arbejdet, når det handler om at skabe innovation, og hvad der kræves af individet i ansættelsen. Da innovation kan opleves som et abstrakt begre...

  19. Caryological notes in some portuguese <i>Ranunculaceae>

    Directory of Open Access Journals (Sweden)

    Queirós, Margarida

    1990-05-01

    Full Text Available Chromosome numbers of fourteen portuguese laxa of <i>Ranunculaceae> are reported: <i>Helleborus foetidusi> 20 = 32; <i>Nigella damascenai> 2n = 12; <i>N. gallicai> 2n = 12; <i>Delphinium Pentagynumi> 2n = 16; <i>D. Halteratumi> subsp. <i>verdunense> 2n = 16; <i>Anemone palmetai> 2n = 32; <i>Clematis campaniflorai> 2n = 16; <i>Ranunculus muricatusi> 2n = 48; <i>R. repensi> 2n = 32; <i>R. bulbosusi> subsp. <i>aleae> var. <i>adscendens> 2n =16; <i>R. sceleratusi> 2n = 32; <i>R. paludosusi> 2n = 32; <i>R. nigrescensi> 2n = 16; <i>Aquilegia vulgarisi> subsp. <i>dichroa> 2n = 14. The chromosome numbers are in accordance with previous results.

    Se estudia el número cromosómico de algunos táxones de <i>Ranunculaceae> portugueses: <i>Helleborus foetidusi> 2n = 32; <i>Nigella damascenai
    > 2n = 12; <i>N. gallicai> 2n = 12; <i>Delphinium Pentagynumi> 2n = 16; <i>D. Halteratumi> subsp. <i>verdunense> 2n = 16; <i>Anemone palmetai> 2n = 32; <i>Clematis campaniflorai> 2n = 16; <i>Ranunculus muricatusi> 2n = 48; <i>R. repensi> 2n = 32; <i>R. bulbosusi> subsp. <i>aleae> var. <i>adscendens> 2n =16; <i>R. sceleratusi> 2n = 32; <i>R. paludosusi> 2n = 32; <i>R. nigrescensi> 2n = 16; <i>Aquilegia vulgarisi> subsp. <i>dichroa> 2n = 14. Estos recuentos coinciden con los obtenidos anteriormente por otros autores.

  20. I zhizn, i sljozõ, i ljubov

    Index Scriptorium Estoniae

    2005-01-01

    30. sept. kuni 6. okt. Tallinnas toimuvast I Vene Kino festivali sügissessioonist. Kolme programmi (Põhiprogramm, Rahvalik kino, Laste- ja noorteprogramm) raames näidatavate filmide tutvustused-soovitused

  1. How I became what I am

    Indian Academy of Sciences (India)

    Lawrence

    Iwas born into a highly educated family in Kolkata on a cold Decem- ber afternoon ... to spend quality time with me, and with her encouragement and sup- port, I grew up ... career at ICAR meant that he spent much of his working life outside. Kolkata, in ... lieve I should continue with my work in fulfilling her goals. My work on ...

  2. Reminiscences by Dr. I.I. Rabi

    International Nuclear Information System (INIS)

    Rabi, I.I.

    1973-01-01

    Dr. I.I. Rabi, Professor of Physics, Columbia University, Nobel Laureate, adviser to presidents and an original member of the scientific advisory committees both of the United Nations and the IAEA, delivered the following address at the Salazar Atomic Centre, Mexico, in October 1972 he spoke on 'reminiscences from scientific advisory services to governments and international organizations'. (author)

  3. Livet i skolen - skolen i livet

    DEFF Research Database (Denmark)

    Rasmussen, Lisa Rosén

    og sanset skolehverdag. Med poststrukturalistiske begreber om tidslighed og samtidighed diskuteres, hvordan fortiden forstyrrer og intensiveres i nutiden. I analyser af erindringernes både sproglige, materielle og affektive dimensioner trækkes på teorier af bl.a. Judith Butler, Karen Barad, Eelco...

  4. <i>Delphinium> L. Subgen. <i>Delphinium>: origin and evolutionary trends

    Directory of Open Access Journals (Sweden)

    Blanché, Cèsar

    1990-12-01

    Full Text Available The advanced features of <i>Delphinium> lo subgen. <i>Delphinium>(annual taxa are compared with those of subgen. <i>Delphinastrum> (DC. Wang and subgen. <i>Oligophyllon> Dimitrova (perennial taxa. F1ower morphology shows a functional interchange between lateral petals and upper petals. and inflorescence structure favours geitonogamy-autogamy rates. Karyotype evolution is based on a decrease in the total length of chromosomes and on an increase in the degree of asymmetry: the chromosome number remains constant (2n = 16 in the subgen. <i>Delphinium>.The dispersal efficiency of the annual species is higher than that of perennials due to seed set increase and improved floatability. both in water and in air. Other advanced adaptive features are the acquisition or new chemical weapons and the emergence of a new embryonogenic type. The ecological niches or subgen. <i>Delphinium> correspond to open and disturbed habitats in contrast to the stable and relatively closed ones or subgenera <i>Delphinastum> and <i>Oligophyllon>. A final hypothesis is made concerning the evolutionary trends observed in annuals vs. perennials in connexion with biogeographical considerations, and a final taxonomic summary is given.

    [ca] Les característiques avançades de <i>Delphinium> L. subgèn. <i>Delphinium> (tàxons anuals són comparades amb les del subgèn. <i>Delphinastrum> (DC. Wang i del subgèn. <i>Oligophyllon> Dimitrova (tàxons perennes. La morfologia norai mostra un intercanvi de funcions entre els pètals laterals i els pètals superiors i l'estructura de la inflorescència de molts tàxons anuals afavoreix un augment de les taxes de geitonogàmia-autogàmia. L'evolució dels cariotips és basada en una disminució de la longitud total dels cromosomes i en un increment del grau d'asimetria; el nombre cromosòmic roman constant per a totes les espècies anuals (2n = 16. l'eficàcia de la dispersió de les esp

  5. Nye Byggesten i Byfornyelsen

    DEFF Research Database (Denmark)

    Ulfstjerne, Michael Alexander

    Publikationen ’Nye Byggesten i Byfornyelsen’ henvender sig primært til kommunale praktikere, der arbejder indenfor området af boligplacering og integration af ygtninge. Vi tager afsæt i det faktum, at grupper af mennesker placeres - i første omgang midlertidigt - i danske yderområder. Publikation...

  6. Stress i gymnasiet

    DEFF Research Database (Denmark)

    Nielsen, Anne Maj; Lagermann, Laila Colding

    Denne undersøgelse af stress hos gymnasieelever i Aalborg viser, hvordan stress giver sig udslag i gymnasiet, hvad der stresser eleverne, hvad der adskiller de stressramte elever fra andre elever, hvordan et stressreduktionskurset Åben og Rolig for Unge virker for de unge i gymnasiet, og hvad der...... kan modvirke stress i gymnasiet....

  7. Gerontologi i uddannelserne

    DEFF Research Database (Denmark)

    Glasdam, Stinne; Jacobsen, Frode Fadnes

    2011-01-01

    Artiklen viser, hvordan gerontologi er lavt prioriteret i uddannelser på bachelor-, diplom- og masterniveau (kandidat) i primært Danmark og Norge. Der tages udgangspunkt i et nordisk kortlægningsarbejde, som blev gennemført i 2008. Dette suppleres med danske og norske aktiviteter, som er kommet til...

  8. I had a dream

    Institute of Scientific and Technical Information of China (English)

    By William E. Wright

    2012-01-01

    I had a dream. Like Martin Luther King, Jr. said in a speech memorized by almost all Chinese students, I had a dream. My dream was that one day I would teach in a foreign country. Little did I know how fascinating and rich teaching in the Law School of Shandong University would be.

  9. FILM I KUFFERTEN

    DEFF Research Database (Denmark)

    Johansen, Stine Liv; Hansen, Adriana Maria

    2013-01-01

    kulturen, medierne er i kulturen – også i legekulturen. Denne rapport beskriver og undersøger et særligt eksempel på medialiseret leg. På baggrund af feltstudier i fem danske børnehaver, hvor et nyt filmpædagogisk materiale – Film i Kufferten - introduceres, er det rapportens formål at eksemplificere...

  10. Analysis of a child infected with [i]Hymenolepis[/i] [i]diminuta[/i] in Poland

    Directory of Open Access Journals (Sweden)

    Przemysław Kołodziej

    2014-09-01

    Full Text Available [i]Hymenolepis diminuta[/i] is a cosmopolitan parasite of rats and mice which is very rare in humans. This study presents the case of a 3-year-old boy infected with [i]Hymenolepis diminuta[/i] in Poland. The diagnosis was based on eggs found and their morphology in the patient’s stool.

  11. Offentlighed i Norden

    DEFF Research Database (Denmark)

    Jørgensen, Oluf

    Offentlighed i Norden forklarer og sammenligner retsreglerne for acces til dokumenter og data i Sverige, Finland, Danmark, Norge og Island. Internationale regler fra FN, Europarådet og EU, der har stigende betydning, bliver også belyst. Offentlighed i Norden viser, at offentlighedsreglerne i de fem...

  12. Ergoterapi i Oman

    DEFF Research Database (Denmark)

    Pedersen, Ulla

    2000-01-01

    En historie om at forstå et land på dets egne præmisser. Refleksioner over sundhedsvæsenet i Oman kontra i Danmark.......En historie om at forstå et land på dets egne præmisser. Refleksioner over sundhedsvæsenet i Oman kontra i Danmark....

  13. Brugerskabte data i bibliotekskatalogen

    DEFF Research Database (Denmark)

    Neumann, Ira

    2008-01-01

    I masterafhandlingen diskuteres begrebet folksonomi, dels ud fra den foreliggende litteratur på området, dels på baggrund af en empirisk undersøgelse af tags i Ann Arbor District Library’s katalog. Med udgangspunkt i Roman Jakobsons model over sproglige funktioner i en kommunikationsakt bliver det...

  14. Augmented Reality i naturfagsundervisningen

    DEFF Research Database (Denmark)

    Radmer, Ole; Surland, Mogens; Nielsen, Birgitte Lund

    Augmented Reality (AR) giver ny mulighed for, at elever kan lave undersøgelser i naturfag med enkel teknologi, hvor animationer og simulationer kobles med det virkelige fænomen. I workshoppen kan I afprøve AR eksempler, udviklet i et internationalt EU projekt. Der vil være noget, der direkte kan...

  15. Menneskerettigheder i professionsperpektiv

    DEFF Research Database (Denmark)

    Eliassen, Gry

    2016-01-01

    Skolepædagoger og -lærere skal kunne identificere og håndtere menneskeretslige udfordringer i undervisningen og i praksis i skolerne. Derfor har Institut for Menneskerettigheder i samarbejde med lektorer fra læreruddannelserne ved Metropol, UC SYD, UCC og UCSJ udviklet nyt undervisningsmateriale,...

  16. Relationer i skolen

    DEFF Research Database (Denmark)

    Der har i de senere år været en stigende interesse for relationer eller relationstænkning i pædagogiske sammenhænge. På baggrund af denne interesse præsenteres i denne antologi en række nye artikler, hvis omdrejningspunkt er relationens rolle i læring og livet i skolen. Bogen henvender sig til fa...

  17. Transition probabilities for the alkali isoelectronic sequences Li I, Na I, K I, Rb I, Cs I, FR I

    International Nuclear Information System (INIS)

    Lindgard, A.; Nielsen, S.E.

    1977-01-01

    Dipole transition probabilities, oscillator strengths, lifetimes (mean lives), and branching ratios derived from a numerical Coulomb approximation are presented for experimentally identified (and some extrapolated) states n< or =12, l< or =4 for each of the following members of the alkali sequences (Z/sub net/ is the net charge of the corresponding ion): Li I Z/sub net/=1-15, 17-24 Rb I Z/sub net/=1-6 Na I Z/sub net/=1-24 Cs I Z/sub net/=1-5 K I Z/sub net/=1-7 Fr I Z/sub net/=2,4. The results are presented in transition diagrams and in tables giving energy-level values and transition wavelengths as well. An appendix on hydrogen results for 5< or =n< or =12, 4< or =l< or =11 is included to represent the high-angular-momentum states of all members of the alkali isoelectronic sequences

  18. Flight Deck I-Glasses, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — Flight Deck i-Glasses is a color, stereoscopic 3-D display mounted on consumer style eye glass frames that will enhance operator performance and multi-modal...

  19. Regnorme i salat

    DEFF Research Database (Denmark)

    Krogh, Paul Henning

    Rapporten er en undersøgelse af regnorme som går op i Iceberg salat. Der gennemgås dyrkningsteknikker og andre foranstaltninger, som kan forhindre orm i salathoveder. I en pilotundersøgelse er regnormenes antal for hver art pr. kvadratmeter bestemt for at få et overblik over artsammensætningen i ...... jorden sammenholdt med arten der kravler op i salat. Artsbestemmelsen er understøttet af DNA-barcoding af orme indsamlet fra salathoveder. I rapporten præsenteres baggrunden for anbefalinger af kulturtekniske foranstaltninger baseret på litteratur og ekspertvurderinger....

  20. Kristensen i Hvidovre

    DEFF Research Database (Denmark)

    Vindum, Kjeld

    2001-01-01

    Det er ikke blot lykkedes at tilpasse et rationelt industrialiseret byggeri til strengt funktionelle behov, men også at udforme det med stor følsomhed i forhold til de menneskelige behov i øvrigt om boligbebyggelse i Hvidovre. Alligevel er dele af den truet af nedrivning.......Det er ikke blot lykkedes at tilpasse et rationelt industrialiseret byggeri til strengt funktionelle behov, men også at udforme det med stor følsomhed i forhold til de menneskelige behov i øvrigt om boligbebyggelse i Hvidovre. Alligevel er dele af den truet af nedrivning....

  1. HOPE udveksling i Europa

    DEFF Research Database (Denmark)

    Pedersen, Ulla

    1996-01-01

    En artikel om deltagelse i HOPE (Hospital Professional Exchange) - 6 ugers studiebesøg på et hospital i udlandet. Ulla Pedersen fortæller om 6 uger med fokus på rehabilitering i det hollandske sundhedsvæsen.......En artikel om deltagelse i HOPE (Hospital Professional Exchange) - 6 ugers studiebesøg på et hospital i udlandet. Ulla Pedersen fortæller om 6 uger med fokus på rehabilitering i det hollandske sundhedsvæsen....

  2. Systems of Rb2I2-CdI2-PbI2 and Cs2I2-CdI2-PbI2

    International Nuclear Information System (INIS)

    Volchanskaya, V.V.; Il'yasov, I.I.

    1979-01-01

    The Rb 2 I 2 -CdI 2 -PbI 2 and Cs 2 I 2 -CdI 2 -PbI 2 triple systems have been studied, using the visual-polythermal method. The liquidus of the systems researched consists of the components and compounds crystallization fields: 2RbIxCdI 2 , 2RbIxRbI 2 , RbIxPbI 2 and 2CsIxCdI 2 , 4CsIxPbI 2 , CsIxPbI 2 , respectively. The crystallization fields converge in four non-variant points at 360, 280, 205 and 192 deg C in the Rb 2 I 2 -CdI 2 -PbI 2 system and at 375, 368, 208 and 190 deg C in the CsI 2 -CdI 2 -PbI 2 system

  3. Friluftsliv i Danmark

    DEFF Research Database (Denmark)

    Andkjær, Søren

    semistrukturerede interviews med deltagere og ledere i friluftsliv fra de udvalgte cases. Herudover inddrages struktureret og direkte deltagende observation af praksis samt dokumentmateriale. I den komparative, perspektiverende del, er der foretaget et kvalitativt casestudy af Outdoor Education i New Zealand med...... anvendelse af metoder svarende til den kvalitative undersøgelse af organiseret friluftsliv i Danmark. Analysen af Outdoor Education i New Zealand tjener primært til at give perspektiv til undersøgelsen af friluftsliv i Danmark.   Resultater Deltagelse i friluftsliv handler for de unge primært om sociale...... til udtryk i from af undervisernes pædagogiske idealer samt skolernes værdisæt, og i mindre grad er en del af deltagernes oplevelse og forståelse. Praksis og værdier i Outdoor Education i New Zealand kan på mange måder sammenlignes med adventure, som en nyere trend i friluftsliv i Danmark. Samtidigt...

  4. Etik i fysioterapi

    DEFF Research Database (Denmark)

    Præstegaard, Jeanette

    2001-01-01

    Et kvalitativt interviewstudie af en gruppe fysioterapeuters etiske perception i forhold til etiske problemstillinger opstået i daglig praksis samt en etisk analyse af Danske Fysioterapeuters etiske retningslinier...

  5. Mindfulness i undervisningen

    DEFF Research Database (Denmark)

    Lau, Dorthe

    2012-01-01

    Hverdagen i skole og institutioner er fyldt med udfordringer og krav om læring og udvikling i fællesskabet. Dette stiller store krav til den enkelte, og de mange relationer i løbet af en dag. Dette kan i nogle tilfælde fjerne fokus fra den enkeltes evne til nærvær både i forhold til sig selv og i...... forhold til andre. Mindfulness i undervisningen er et redskab til at skabe et lærings- og udviklingsmiljø, hvor børn får erfaringer med at være til stede i sig selv, og hvor de oplever ro til at lære og være....

  6. Smart Energi i Hjemmet

    DEFF Research Database (Denmark)

    Jensen, Ole Michael

    Med denne rapport foreligger en evaluering af det såkaldte SEIH-projekt: Smart Energi i Hjemmet. Projektet er gennemført i samarbejde med 191 husejere i Middelfart Kommune med formål at afsøge mulighederne for at opnå energibesparelser i enfamiliehuse ved at bruge automatik til at sænke temperatu......Med denne rapport foreligger en evaluering af det såkaldte SEIH-projekt: Smart Energi i Hjemmet. Projektet er gennemført i samarbejde med 191 husejere i Middelfart Kommune med formål at afsøge mulighederne for at opnå energibesparelser i enfamiliehuse ved at bruge automatik til at sænke...

  7. iComplaints

    Data.gov (United States)

    US Agency for International Development — iComplaints is a web-based Equal Employment Opportunity (EEO) complaint tracking and reporting system. The Office of Equal Opportunity Programs uses iComplaints to...

  8. I den sidste time

    DEFF Research Database (Denmark)

    Norup, Lis

    : Dekadencens teori. Arthur William Symons: Dekadencens litteratur. Oscar Wilde: Kritikeren som kunstner) Noter I tekster skrevet i 1800-tallet udfoldes dekadencekunsten som den forfinede sensibilitets, de forstyrrede sansers og det oprevne sinds digtning. Med litteraturhenvisninger....

  9. Radon i danske lejeboliger

    DEFF Research Database (Denmark)

    Rasmussen, Torben Valdbjørn; Skytte Clausen, Louise

    I denne undersøgelse kortlægges radonindholdet i indeluften og det undersøges, hvordan indholdet af radon i indeluften er fordelt og spredes i en ejendom, og om det er muligt at pege på en bygningsdel eller en bygningskomponent som en spredningsvej for radon i boliger. Boligerne er lejeboliger og...... ligger i etageejendomme, kæde- og rækkehuse tilhørende bygningstyper opført fra 1850 og frem. De udvalgte ejendomme ligger i områder af landet, hvor der ved tidligere undersøgelser har vist sig at være en stor andel af huse med et højt indhold af radon i indeluften. Koncentrationen af radon er målt over...

  10. Madkultur i skolen

    DEFF Research Database (Denmark)

    Brock, Steen; Christensen, Anne-Marie Søndergaard

    2016-01-01

    Artiklen belyser etiske problemstillinger omkrig dette at spise i skoletiden, på baggrund af Jürgen Habermas og Ludwig Wittgensteins respektive moralopfattelser. I særlig grad tematiseres forholdet mellem medborgerskab og verdensborgerskab, samt etikkens eksistentielle dimensioner...

  11. Should I Gain Weight?

    Science.gov (United States)

    ... Videos for Educators Search English Español Should I Gain Weight? KidsHealth / For Teens / Should I Gain Weight? ... something about it. Why Do People Want to Gain Weight? Some of the reasons people give for ...

  12. Inquiry i naturfags undervisningen

    DEFF Research Database (Denmark)

    Harlen, Wynne; Guldager, Ida; Auning, Claus

    Hvordan arbejde med en IBSE tilgang til undervisningen og beskrivelse af erfaringer fra Fibonacci Projektet i Danmark......Hvordan arbejde med en IBSE tilgang til undervisningen og beskrivelse af erfaringer fra Fibonacci Projektet i Danmark...

  13. Med literacy i bagagen

    DEFF Research Database (Denmark)

    Larsen, Kirsten Friis

    2016-01-01

    Dette kapitel præsenterer kort literacybegrebet, og det fremhæver, hvorfor læringsmål er så vigtige for barnets literacykompetencer og literacylyst – både i hjemmet, i dagtilbuddet og i børnehaveklassen. Tankerne bag denne bog introduceres således her. Indholdet i de øvrige kapitler beskrives des...

  14. NY KANDIDATUDDANNELSE I LYSDESIGN

    DEFF Research Database (Denmark)

    Hansen, Ellen Kathrine; Mullins, Michael

    2013-01-01

    En kandidatuddannelse på højt internationalt niveau i lysdesign har været drøftet og ønsket gennem mange år blandt branchens aftagere. Der er en bred enighed om, at der i Danmark er behov for en tværfaglig uddannelse i lysdesign. Aalborg Universitet, Institut for Arkitektur og Medieteknologi har ...... Denmark, ved positiv behandling forventes, at de første stude- rende starter i september 2014....

  15. 131I Metaiodobenzylguanidine scintigraphy

    International Nuclear Information System (INIS)

    Izumi, Motomori; Morimoto, Isao; Yamashita, Shunichi; Hirayu, Hideshi; Nagataki, Shigenobu

    1988-01-01

    A newly developed radiopharmaceutical agent, 131 I-metaiodobenzylguanidine ( 131 I-MIBG) has been reported to be very useful for locating pheochromocytoma and to be specific for pheochromocytoma and safe for humans. The first 131 I-MIBG scintiscanning in Japan which has been carried out in our clinic and the analysis of clinical experience of 131 I-MIBG scanning in Japan are presented

  16. BIM i vejsektoren

    DEFF Research Database (Denmark)

    Kjems, Erik

    2015-01-01

    BIM er et udtryk de fleste er stødt på i forbindelse med deres erhverv. Det er meget udbredt i byggesektoren, men knap så kendt i vejsektoren. I det følgende vil det blive forsøgt at forklare begrebet, baggrunden og indholdet herfor, og hvorfor det er noget alle bør tage stilling til inden for en...

  17. Variation i morfologi

    DEFF Research Database (Denmark)

    Gudiksen, Asgerd

    2011-01-01

    Artiklen gør indledningsvis rede for hvordan de enkelte bøjningsendelser er manifesteret i ømålene og sætter formerne i relation til formerne i gammeldansk. Derefter beskrives verbernes fordeling på 1. svage bøjning (ede-bøjningen) og 2. svage bøjning set i relation til standarddansk, og der...

  18. Supervision som undervisningsform i voksenspecialundervisningen

    DEFF Research Database (Denmark)

    Kristensen, René

    2000-01-01

    Supervision som undervisningsform i voksenspecialundervisningen. Procesarbejde i undervisning af voksne.......Supervision som undervisningsform i voksenspecialundervisningen. Procesarbejde i undervisning af voksne....

  19. Faldseminar i Region Hovedstaden

    DEFF Research Database (Denmark)

    Evron, Lotte Orr

    2017-01-01

    I april deltog jeg i et spændende faldseminar med Videncenter for hjælpemidler og velfærdsteknologi (VihTek) på Rigshospitalet Glostrup, hvor fire teknologier til forebyggelse af fald blev præsenteret. Disse teknologier kan være interessante for sygeplejersker der arbejder i primær sektor. Her vil...

  20. Skriftlige opgaver i sygeplejerskeuddannelsen

    DEFF Research Database (Denmark)

    Glinsvad, Birte; Frederiksen, Kirsten

    2014-01-01

    Kapitlet tager fat i to af de væsentligste forhold, der kendetegner en god sygeplejefaglig opgave: argumentation og struktur. De illustreres gennem et eksempel på en type opgave, som studerende typisk vil møde i starten af uddannelsen. Kapitlet afsluttes med Stephen Toulmins argumentationsmodels...... argumenter i projektet og projektet set som ét overordnet argument....

  1. Ulighed i sundhed

    DEFF Research Database (Denmark)

    Uligheden i sundhed er stigende i Danmark trods et mangeårigt politisk fokus på at bekæmpe den. Bogen bidrager med viden om de komplekse processer, hvori ulighed i sundhed opstår og reproduceres. Bogens kapitler handler bl.a. om: Mænd og sundhed Klasse, sundhed og subjekt Overvægt Mental sundhed,...

  2. Markedskommunikation i praksis

    DEFF Research Database (Denmark)

    Markedskommunikation handler om al kommunikation mellem aktørerne på et marked. Derfor er der ofte tale om vidtforgrenede processer, hvor budskaber bliver skabt i samarbejdet mellem aktører og sjældent kan styres af én afsender alene. Markedskommunikation i praksis tager udgangspunkt i denne dyna...

  3. Situeret interesse i biologi

    DEFF Research Database (Denmark)

    Dohn, Niels Bonderup

    2006-01-01

    Interesse hævdes at spille en vigtig rolle i læring. Med udgangspunkt i interesseteori og situeret læring har jeg foretaget et studium i en gymnasieklasse med biologi på højt niveau, med henblik på at identificere hvilke forhold der har betydning for hvad der fanger elevers interesse. Jeg har...

  4. Holmes i danske tegneserier

    DEFF Research Database (Denmark)

    Lauridsen, Palle Schantz

    2017-01-01

    En mediehistorisk og -analytisk undersøgelse af fremstillingen af Sherlock Holmes-karakteren og -universet i tegneserier udgivet i Danmark. Del 1 omhandler en upåagtet række søndagsstriber, Storm P. tegnede for Ekstrabladet i 1910-11. De hører til blandt Danmarks allerførste tegneserier...

  5. It i undervisningen

    DEFF Research Database (Denmark)

    Mathiasen, Helle

    2012-01-01

    Spørgsmålet, hvorfor skal vi bruge IT i undervisningen?, kan i dag umiddelbart besvares med, at vi som borgere skal vænne os til at vores arbejdsliv og alle samfundsmæssige funktioner digitaliseres gennemgribende, samt at lovgivningen kræver, at uddannelsesinstitutioner inddrager IT i undervisnin...

  6. Indvandrere i job

    DEFF Research Database (Denmark)

    Rosdahl, Anders; Skovgaard Møller, Simon

    Som for andre langtidsledige danskere er det svært for langtidsledige med ikkevestlig baggrund at bryde mønsteret og komme i arbejde. Men indvandrere og efterkommere har ofte nogle yderligere problemer i form af fx utilstrækkeligt kendskab til det danske arbejdsmarked og til det danske sprog. I d...

  7. I sandhed freudiansk

    DEFF Research Database (Denmark)

    Bonde, Lisbeth

    2015-01-01

    Lucian Freud (1922-2011) går i bogstaveligste forstand i kødet på sine portrætofre. Ligesom sin berømte farfar, Sigmund Freud, som grundlagde psykoanalysen, interesserer han sig for den menneskelige eksistens. Men i stedet for på et videnskabeligt grundlag at kortlægge forpinte menneskers indre...

  8. Hvorfor master i medborgerskab?

    DEFF Research Database (Denmark)

    Korsgaard, Ove

    2002-01-01

    Danmarks Pædagogiske Universitet planlægger i samarbejde med Syddansk Universitet at udbyde en master i medborgerskab: etisk og demokratisk dannelse. Artiklens forfatter gør rede for nogle af de tanker, der ligger bag uddannelsen, og belyser, hvorfor medborgerskab er blevet et nøglebegreb i nyere...

  9. Talentudvikling i Sport

    DEFF Research Database (Denmark)

    Talentudvikling er et satsningsområde i Danmark. Det er helt berettiget og dybt nødvendigt. Danmark er et lille land på en stor international scene. Vi skal kunne lave talentudvikling i verdensklasse. Heldigvis gør vi det godt. Dansk talent-forskning er i international top klasse. De bedste dansk...

  10. Klimadebatten i Danmark

    DEFF Research Database (Denmark)

    Danielsen, Oluf

    type klimadebat, der i dag er vokset til et meget stort omfang. Nedslaget ved de to klimatopmøder, Conferences Of Partners, dvs. konferencer med deltagelse af de lande, der i 1992 i Rio de Janeiro vedtog og siden ratificerede FN's Klimakonvention, hænger sammen med en del af projektets gennemgående...

  11. Problemskabende ungdomsgrupper i Danmark

    DEFF Research Database (Denmark)

    Perry, Kevin

    2013-01-01

    Baggrunden for denne rapport er et ønske om at belyse, hvad der kendetegner grupper af unge med problemskabende og kriminel adfærd – som grupper. Undersøgelsens empiri udgøres af interview 12 steder i landet med i alt 55 professionelle fagfolk, der kender og arbejder med de unge i lokalområderne....

  12. Production of 123I

    International Nuclear Information System (INIS)

    Britto, J.L.Q. de; Silva, A.G. da; Teixeira, F.C.M.; Costa e Silva, A.; Souza, A.S.F. de

    1981-01-01

    The 12 I is prepared by 122 Te( 3 He, 2n) 123 Xe sup(β+) → 123 I reaction. The remotization of the Xe diffusion and capture system and the retired of the 123 I produced by the 123 Xe precursory decaying. (E.G.) [pt

  13. Hurtigt i gang

    DEFF Research Database (Denmark)

    Krogh Graversen, Brian; Damgaard, Bodil; Rosdahl, Anders

    hurtigere i job. Det gjaldt for alle grupper af ledige, men effekten var særlig stor for akademikere. Tilsyneladende har mange stærke ledige hurtigt selv fundet et job, for at undgå at deltage i aktiviteterne under den intensive indsats. Svagere ledige synes derimod at være blevet hjulpet i gang af...

  14. Video narrativer i sygeplejerskeuddannelsen

    DEFF Research Database (Denmark)

    Jensen, Inger

    2009-01-01

    I artiklen gives nogle bud på hvordan video narrativer kan bruges i sygeplejerskeuddannelsen som triggers, der åbner for diskussioner og udvikling af meningsfulde holdninger til medmennesker. Det belyses også hvordan undervisere i deres didaktiske overvejelser kan inddrage elementer fra teori om...

  15. Teorin i praktiken - praktiken i teorin

    DEFF Research Database (Denmark)

    Lund, Birthe; Lindhart, Lars

    2006-01-01

    Vad ligger bakom våra föreställningar om "teori" och "praktik"? Är de varandras motsatser och oförenliga? Eller är de aspekter både i lärande och yrkesutövande? I boken talas om den "praktikchock" som ofta möter blivande lärare när de är ute i skolornas "verklighet" för första gången. Denna antol...... antologi med alla sina lärda, inspirerande och insiktsfulla bidrag vänder sig till verksamma lärare och lärarstuderande som närmar sig slutet av sin utbildning och alla andra som arbetar med mötet mellan teori och praktik....

  16. Tablets i skolen

    DEFF Research Database (Denmark)

    Lorentzen, Rasmus Fink

    2012-01-01

    Denne rapport afslutter CELMS undersøgelse af Odder Kommunes projekt med indførelse af iPads på alle kommunens skoler. Undersøgelsen har til formål at belyse om der er pædagogiske og læringsmæssige fordele forbundet med brugen af tablets i undervisningen i grundskolen og i givet fald hvilke...... designer og tablet’ens egenskaber i et generelt perspektiv. Rapporten afsluttes med en række anbefalinger til henholdsvis lærere og skoleledere med henblik på videre udvikling af indsatsen....

  17. Virkeligheder i Second Life

    DEFF Research Database (Denmark)

    Jensen, Sisse Siggaard

    "Virkeligheder i Second Life" er en beretning, der bygger på tre case studier samt omfattende observationer i den virtuelle verden Second Life i perioden 2006 til 2008. Beretningen er den ene af to bøger der bygger på forskning i virtuelle verdener; de tre case studier er: Wonderful Denmark, en...... virtuel virksomhed, Literary, et virtuelt bibliotek, Times, en afdeling af en real life virksomhed. Den anden bog: "Oplevelser i en virtuel verden" omhandler den virtuelle verden EverQuest - et masserollespil....

  18. Jernkarbonatutfelling (Sideritt) i myr

    OpenAIRE

    Selmer-Olsen, A. R.; Lie, Ole

    1983-01-01

    I torva langs det NV utløpet fra Glesmyra er det funnet et 15 - 20 cm tykt lyst grått sjikt l, 5 m nede i profilet. Aldersbestemmelse, røntgenbestemmelse og kjemiske analyser viser at dette er jernkarbonat (sideritt) dannet for 5700 år siden. Det er diskutert hvordan dette sjiktet kan ha blitt dannet. Som konklusjon, har en kommet fram til at det har vært en utfelling av jernhydroksyd i bekkeløpet. I løpet av et visst tidsrom har pH i vannet steget noe ved at jernet ha blitt redusert og hydro...

  19. Identitetspolitik i klasserummet

    DEFF Research Database (Denmark)

    Buchardt, Mette

    Afhandlingen er et kombineret uddannelses- og videnssociologisk anlagt studie af klasserummets curriculum. Nærmere bestemt et studie af identitetspolitikker knyttet til 'religion' og 'kultur', som disse udformer sig i klasserummet i relation til vidensproduktion og social klassifikation. Kategorier...... som 'muslim' og 'dansk' søges dermed opbrudt i et studie af klasserummet som rum for vidensproduktion og produktion af social forskel. Hvilken viden om religion produceres? Hvilke pladser for subjekter/subjektiviteter? Hvilke måder at være elev på? Og hvordan indgår 'muslimskhed' og 'danskhed......'/'kristenhed' i klasserummets sociale økonomi? Klasserummet studeres dermed som en mikropolitisk arena for relationer og politikker i forhold til minoriteter og majoritet, og hvordan disse indgår i klasserummets sociale økonomi.   Projektets datamateriale er konstrueret ud fra observation af to afgrænsede...

  20. Hjemme i globaliseringen

    DEFF Research Database (Denmark)

    Mechlenborg, Mette

    2007-01-01

    I nærværende artikel her er det min hensigt at sætte fokus på den aktuelle dyrkelse af hjemmet, som har pustet liv i en diskussion om hjemmets rolle i globaliseringsteorierne. I dag synes udfordringen ikke at forstå globaliseringen overfor det forankrede hverdagsliv, men at forsøge at etablere en...... dialog mellem de to. Ved at sætte begrebet hjem centralt i forhold til teorier om mobilitet, modernisering og globalisering, vil denne artikel sætte fokus på kløften mellem stereotype opfattelser af globaliseringen og indgroede myter om hjem og hverdagsliv. Teoretisk og metodisk er artiklen placeret i...

  1. Moda i antropologija

    Directory of Open Access Journals (Sweden)

    Danijela Velimirović

    2016-03-01

    Full Text Available Želeći da istraživanje odevanja zatalasa i na našim prostorima, uredništvo Etnoantropoloških problema odlučilo je da ovu svesku posveti studijama mode. Uvažavajući interdisciplinarnost ovih studija, ono je okupilo istoričare umetnosti, antropologe, etnologe i teoretičare mode kako bi zajedno razmotrili pitanje relevantnosti ovog fenomena u konstrukciji različitih identiteta (rodnih, seksualnih, klasnih, religijskih ili supkulturnih, te sagledali složene odnose mode i ideologije. Danas veoma aktuelno pitanje uloge i značaja modnih zbirki u muzejima takođe je potaknuto u zborniku.

  2. I'm back

    Science.gov (United States)

    Bill Block

    2012-01-01

    As some of you might know, I am the new Editor-In-Chief for the Journal of Wildlife Management. Just to get acquainted, here are a few tidbits about me. I am the Program Manager for the Wildlife and Terrestrial Ecosystems Science Program with Rocky Mountain Research Station of the U.S. Forest Service. I consider myself a generalist having worked on reptiles, amphibians...

  3. Kompetencer i dansk erhvervsliv

    DEFF Research Database (Denmark)

    Harmsen, Hanne

    2003-01-01

    er bredt og i sine forskellige sammenhænge giver udtryk for den fokus, der i øjeblikket er på medarbejdernes ressourcer eller virksomhedens viden. Og så er det endnu et nyt begreb i en management-verden som med mindre og mindre mellemrum tager nye ledelsesbegreber til sig, som indimellem nærmest får...

  4. Kompetencer i dansk

    DEFF Research Database (Denmark)

    Bundsgaard, Jeppe; Christiansen, Eva Terese; Flamant, Sanne Harder

    udvikler nogle af de væsentligste kompetencer i det senmoderne samfund. Kompetencetilgangen stiller en logik og en metode til rådighed for valg af undervisningsindhold og -metoder og danner desuden grundlag for refleksion i og over undervisningen. Kompetencer i dansk behandler bl.a. emner som litteratur......, kommunikation, sociale netværk, læringsspil, gruppearbejde, evaluering og interkulturelle kompetencer. Til bogen er knyttet hjemmesiden www.kompetenceridansk.dk....

  5. Medier i hele kroppen

    DEFF Research Database (Denmark)

    Johansen, Stine Liv

    2010-01-01

    Små børn forholder sig fra en tidlig alder til både elektroniske medier, legetøj og merchandise i en kropslig og legende praksis. I denne artikel præsenteres resultater fra ph.d. afhandlingen Seere i bleer - små børns møde med medier. Artiklen vil især fokusere på, hvordan børnenes mediebrug kan...

  6. Kinas kapabiliteter i cyberspace

    DEFF Research Database (Denmark)

    Hjortdal, Magnus

    Briefet præsenterer tre årsager til at stater anvender deres evner i cyberspace på en offensiv måde, og viser at cyberspace er – og vil blive ved med at være – et afgørende element i Kinas strategi for opstigningen i det internationale system. De tre årsager er: 1) afskrækkelse via infiltration, ...

  7. i'and.

    African Journals Online (AJOL)

    cr:it.i.cat·'issue. for- maintaining mismanagement can result in -~ (6f increasing) per crilpi~. fo9d ..... proportion' of ·esti'ma-:i;etl a·.ferage . yield for:. the significantly low to justify their indusion '':ir~ the modified: model. The major crops inc.luded in the - model are maize(m) f;:a!?!i~v~ · and vegetatlles(g). · ta@ ___ ~p$ight ...

  8. Kommunisme i London

    DEFF Research Database (Denmark)

    Hansen, Brian Benjamin; Nielsen, Kasper Porsgaard

    2010-01-01

    Begrebet ”kommunisme” var på dagsordenen, da over 800 mennesker mødtes i Birkbeck University College i London, den 13.-15. marts, 2009. En perlerække af talere var inviteret: Alain Badiou, Michael Hardt, Bruno Bosteels, Peter Hallward, Allesandro Russo, Alberto Toscano, Toni Negri, Terry Eagleton......, Jacques Rancière, Slavoj Žižek, Gianni Vattimo og Judith Balso. Her følger en rapport fra begivenheden....

  9. Kildesortering i etageejendomme

    DEFF Research Database (Denmark)

    Petersen, Lars Kjerulf; Kristiansen, Tilde Nikoline Bernth

    2017-01-01

    Rapporten fremlægger resultaterne fra en sociologisk undersøgelse vedrørende kildesortering af husholdningsaffald i etageejendomme. Undersøgelsen viser, at der findes en udbredt norm om at man bør sortere sit affald. Denne norm bliver også i høj grad omsat i praksis, om end det beror på, at der e...

  10. Energikravene i BR15

    DEFF Research Database (Denmark)

    Kragh, Jesper; Aggerholm, Søren

    2015-01-01

    Bygningsreglement 2015 – BR15 – gælder fra 1. januar 2016 med en overgangsfase frem til 1. juli 2016. Det bygger videre på BR10 og de tidligere udmeldte krav til nybyggeriet frem mod 2020. I denne kvikguide får du hurtigt overblik over, hvordan du sikrer, at de bygninger, du er med til at opføre...... eller renovere, lever op til BR15’s energikrav. Disse krav findes primært i reglementets kapitel 7 om energiforbrug og i kapitel 8 om installationer samt i kapitel 6 om indeklima....

  11. Utopi i praksis

    DEFF Research Database (Denmark)

    Berliner, Peter; De Casas, Elena; Christensen, Jeppe Høj

    2014-01-01

    Artiklen fremlægger en analyse af læring i et fredspsykologisk undervisningsforløb i Mexico som en del af opbygning af demokratiske og social resiliente læringsmiljøer fra børnehave til universitet.......Artiklen fremlægger en analyse af læring i et fredspsykologisk undervisningsforløb i Mexico som en del af opbygning af demokratiske og social resiliente læringsmiljøer fra børnehave til universitet....

  12. Integration i naturen

    DEFF Research Database (Denmark)

    Kjeldsen, Lis Reinholdt

    2015-01-01

    Artiklen formidler resultaterne af udviklingsprojektet: Outdoor learning for Integration through Nature and Cultural Encounters, som er et europæisk samarbejde. Undervisere af flygtning og indvandrere kan med fordel anvende naturen og de kulturelle omgivelser i byen i læringsøjemed.......Artiklen formidler resultaterne af udviklingsprojektet: Outdoor learning for Integration through Nature and Cultural Encounters, som er et europæisk samarbejde. Undervisere af flygtning og indvandrere kan med fordel anvende naturen og de kulturelle omgivelser i byen i læringsøjemed....

  13. Lederens udfordringer i 2014

    DEFF Research Database (Denmark)

    Holt Larsen, Henrik

    2014-01-01

    Du skal i juleferien forberede dig på disciplinen om at vise tillid, være nærværende på afstand, involvering og løbende reflektere over egen lederudvikling. I artiklen peger professor Henrik Holt Larsen på seks aktuelle ledelsesudfordringer for 2014.......Du skal i juleferien forberede dig på disciplinen om at vise tillid, være nærværende på afstand, involvering og løbende reflektere over egen lederudvikling. I artiklen peger professor Henrik Holt Larsen på seks aktuelle ledelsesudfordringer for 2014....

  14. Nyheder i Japan

    DEFF Research Database (Denmark)

    Sejrup, Jens

    2016-01-01

    Kan man stole på, hvad folk siger, når man ikke ved, hvem de er? Forholdet mellem kildeanonymitet og troværdighed er forskelligt i mediesystemer rundt om i verden. Troværdighed er ingen universel størrelse, men en retorisk effekt der opstår på baggrund af faktorer som er kulturelt og systemisk in...... indlejret. I Japan har seriøse nyhedsmedier et specielt forhold til udsagn fra anonyme kilder, og udstrakt brug af uidentificerede informanter står ikke i modsætning til journalistisk kvalitet og pålidelighed....

  15. Sprogbrug i praksis

    DEFF Research Database (Denmark)

    Axel, Erik

    2006-01-01

    Artiklen er et argument for at se på sprogbrug i stedet for sprog. Vi bruger sproget til at meddele os. Derfor må vi i vor forståelse af sprogbrug indbefatte brugere, sprogbrug og betydning. Der tages udgangspunkt i betydninger som et aspekt af praksis, og argumenteres for, at sproget ikke har be......, Potter og Wetherells. Endelig introduceres Roy Harris integrative lingvistik, der argumenterer for, at sprogbrugen må forstås som en samlet kommunikationssituation. Med udgangspunkt i Harris tilgang skitseres en praksisbaseret forståelse af sprog som redskab.  ...

  16. Skole i verdensklasse

    DEFF Research Database (Denmark)

    Andersen, Frans Ørsted

    2013-01-01

    Kronikken er en kommentar og ros til de gode danske PIRLS og TIMSS resultater offentliggjort i december 2012. Der argumenteres for at der tages hensyn til den registrerede fremgang i de igangværende reformprocesser vedr folkeskole og læreruddannelse.......Kronikken er en kommentar og ros til de gode danske PIRLS og TIMSS resultater offentliggjort i december 2012. Der argumenteres for at der tages hensyn til den registrerede fremgang i de igangværende reformprocesser vedr folkeskole og læreruddannelse....

  17. Category I structures program

    International Nuclear Information System (INIS)

    Endebrock, E.G.; Dove, R.C.

    1981-01-01

    The objective of the Category I Structure Program is to supply experimental and analytical information needed to assess the structural capacity of Category I structures (excluding the reactor cntainment building). Because the shear wall is a principal element of a Category I structure, and because relatively little experimental information is available on the shear walls, it was selected as the test element for the experimental program. The large load capacities of shear walls in Category I structures dictates that the experimental tests be conducted on small size shear wall structures that incorporates the general construction details and characteristics of as-built shear walls

  18. Photovoltaic Wire, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — This Small Business Innovation Research Phase I project will investigate a new architecture for photovoltaic devices based on nanotechnology: photovoltaic wire. The...

  19. Seguridad <i>Versus> Libertad

    Directory of Open Access Journals (Sweden)

    Marsal Muntalá, Jordi

    2005-01-01

    Full Text Available <i>El nuevo escenario que se ha abierto con los atentados del 11 de septiembre y la amenaza de las redes del terrorismo islamista yihadista ha planteado nuevamente la relación entre seguridad y libertad en las sociedades democráticas. El papel de los servicios de inteligencia en la prevención de las amenazas contra nuestra seguridad y nuestras libertades y derechos ha sido y seguirá siendo fundamental. Las especiales características de estos servicios que deben obtener suficiente información para elaborar la inteligencia necesaria para que los gobiernos tomen decisiones requiere una legislación adecuada para garantizar eficacia para la seguridad y respeto a las libertades y derechos de los ciudadanos.…i>

  20. Taksonomi i et hotspot

    DEFF Research Database (Denmark)

    Balslev, Henrik

    2016-01-01

    Op mod halvdelen af alle jordens plantearter findes koncentreret på kun 1,4 % af jordoverfladen. Langt de fleste af disse hotspots for biodiversitet befinder sig i de tropiske egne, hvor vores viden om antallet af arter er mangelfuld. Thailand er placeret i centrum af jordens største hotspot, som...

  1. Coaching i perspektiv

    DEFF Research Database (Denmark)

    Bogen er en grundbog, der sætter coaching ind i et større perspektiv og en bredere sammenhæng.......Bogen er en grundbog, der sætter coaching ind i et større perspektiv og en bredere sammenhæng....

  2. Elefanter i bur

    DEFF Research Database (Denmark)

    Sandøe, Peter

    2016-01-01

    Nogle elefanter i zoologiske haver har svært ved at formere sig, og de bruger store dele af dagen på blot at svaje fra side til side. Det kan være tegn på, at de store dyr mistrives i fangenskab....

  3. Informationsarkitektur i retorisk perspektiv

    DEFF Research Database (Denmark)

    Hasle., Per F. V.

    2006-01-01

    Når et nyt vidensgelt etableres, foregår dette normalt med en vis teoretisk forvirring i starten. De to konferencer, som Danmarks Forskningsbiblioteksforenings forum for informationsarkitektur (IA) har afholdt i 2004 og 2005 (se http://www.dfdf.dk/arrangementer_df.php?old=ja) har da også afspejlet...

  4. Portfolio i erhvervsuddannelserne

    DEFF Research Database (Denmark)

    2008-01-01

    Materialet kombinerer korte film med introducerende tekster og belyser fra forskellige vinkler, hvordan portfolio kan bruges som evalueringsmetode i erhvervsuddannelserne. Udgiver: Undervisningsministeriet Udgivelsessted: Pub.uvm.dk......Materialet kombinerer korte film med introducerende tekster og belyser fra forskellige vinkler, hvordan portfolio kan bruges som evalueringsmetode i erhvervsuddannelserne. Udgiver: Undervisningsministeriet Udgivelsessted: Pub.uvm.dk...

  5. Ramanujan and I 1

    Indian Academy of Sciences (India)

    about my connections with Sivasankaranarayana Pillai whom I expected to meet in 1950 in. Cambridge, USA, at the International Congress of Mathematicians. Unfortunately he never arrived because his plane crashed near Cairo. I first heard ofPillai in connection with the following result which he proved: Let f (n) denote ...

  6. Energiforbruget i 6 bygninger

    DEFF Research Database (Denmark)

    Johnsen, Kjeld

    Rapporten omhandler en forundersøgelse af energiforbrugets fordeling på anlæg og komponenter i 6 bygninger: et omsorgscenter, en kontorbygning, et supermarked og tre skoler. Undersøgelsen er et led i arbejdet på at tilvejebringe et grundlag for energiøkonomisk planlægning af nye bygninger og...

  7. Frygt i en ebolatid

    DEFF Research Database (Denmark)

    Rasborg, Klaus

    2014-01-01

    Kriminaliteten er faldende, og der er større risiko for at blive ramt af lynet end ebola. Alligevel stiger frygten i samfundet.......Kriminaliteten er faldende, og der er større risiko for at blive ramt af lynet end ebola. Alligevel stiger frygten i samfundet....

  8. Transportforskning i EU

    DEFF Research Database (Denmark)

    Leleur, Steen

    1996-01-01

    Artiklen giver en gennemgang af aktuelle EU transportforskningsemner samt nogle synspunkter og erfaringer baseret på deltagelse i EU forskningsprojekter.......Artiklen giver en gennemgang af aktuelle EU transportforskningsemner samt nogle synspunkter og erfaringer baseret på deltagelse i EU forskningsprojekter....

  9. Natursyn. Sandheden i naturen

    DEFF Research Database (Denmark)

    Holm, Henrik Ole

    2015-01-01

    Analyse af Eckersbergs natursyn i forhold til 1800-tallets idealistiske filosofi, særlig Immanuel Kant, tilsat post- og dekoloniale overvejelser over vestlig selvforståelse.......Analyse af Eckersbergs natursyn i forhold til 1800-tallets idealistiske filosofi, særlig Immanuel Kant, tilsat post- og dekoloniale overvejelser over vestlig selvforståelse....

  10. Elever i erhvervsuddannelse

    DEFF Research Database (Denmark)

    Gottlieb, Susanne; Götzsche, Hanne

    Skole-virksomhedssamspillet er helt centralt i erhvervsuddannelserne, og med eud-reformen er der kommet to nye elementer, som kan medvirke til en kvalificering af samspillet, nemlig elevens personlige uddannelsesplan og uddannelsesbogen.I dette temahæfte præsenteres erfaringerne fra en undersøgel...

  11. Lege i det fri

    DEFF Research Database (Denmark)

    Ravn, Jakob Iversen

    2007-01-01

    At være et legende menneske fordrer en rettethed mod noget, man kan være legende i forhold til. Eksempelvis en aktivitet, hvor det legende menneske går op i aktiviteten for aktivitetens egen skyld. Overvejelser over friluftslivets legende muligheder...

  12. Can I Prevent Acne?

    Science.gov (United States)

    ... for Educators Search English Español Can I Prevent Acne? KidsHealth / For Teens / Can I Prevent Acne? Print en español ¿Puedo prevenir el acné? What Causes Acne? Contrary to what you may have heard, acne ...

  13. Universelt design i renovering

    DEFF Research Database (Denmark)

    Ryhl, Camilla; Pedersen, Lars Schmidt

    2015-01-01

    Boligbebyggelserne fra 1940'erne og 1950'erne udtrykker gennem funktionalistiske kerneværdier velfærdsstatens demokratiske fundament om social inklusion og ligeværdighed. Disse værdier er også helt centrale i begrebet Universelt design, der i denne artikel beskrives som strategi for øget lighed og...

  14. Kiropraktikkens historie i Danmark

    DEFF Research Database (Denmark)

    Jørgensen, Per

    Bogen er den første samlede, forskningsbaserede fremstilling om kiropraktikkens danske historie. Den har udblik til kiropraktikkens historie i USA.......Bogen er den første samlede, forskningsbaserede fremstilling om kiropraktikkens danske historie. Den har udblik til kiropraktikkens historie i USA....

  15. Acute dental pain I

    DEFF Research Database (Denmark)

    Närhi, Matti; Bjørndal, Lars; Pigg, Maria

    2016-01-01

    Den specialiserede anatomi i pulpa-dentin-organet samt den rige pulpale innervation fra trigeminusnerven forklarer de forskellige typer af smertefølelser i en tand. En kort skarp smerte er typisk for en A-(nerve) fibermedieret smerte, imens en langvarig, bankende smerte indikerer C-(nerve) fibera...

  16. Tvangsauktioner i Danmark

    DEFF Research Database (Denmark)

    Haldrup, Karin; Staunstrup, Jan Kloster; Zangenberg Hansen, Jonas

    Aalborg Universitet og DREAM har analyseret 12.865 boliger solgt på tvangsauktion i perioden 2007-12. Desuden er der foretaget detaljerede analyser af 3.606 tvangsauktionssager i de tre retskredse Roskilde, Næstved og Nykøbing Falster. Disse kredse omfatter 14 kommuner, hvoraf mange har været sær...

  17. Spildesign i Serious Games

    DEFF Research Database (Denmark)

    Helms, Niels Henrik; Uhre Rahbek, Jess

    2012-01-01

    I dette bidrag diskuteres problemfeltet ”didaktisk spildesign” i forhold til principper for godt spildesign og didaktisk design. På baggrund af forskellige forskningsbidrag og udviklingen af et spilelement til støtte for behandlingen af veteraner med PTSD undersøges forholdet mellem spil og lærin...

  18. Stemmer i italiensk litteratur

    DEFF Research Database (Denmark)

    os med sine mange modulationer ind i tekstuniverset og bringer os samtidig tæt på tekstens afsender. Stemmen er ikke kun indhold, men også lyd og tonalitet, herunder flerstemmigheder og tavsheder. Nærværende festskrift er tilegnet Lene Waage Petersen, ekspert i italiensk litteratur. Med stemmer som...

  19. Inklusionsindsatser i folkeskolen

    DEFF Research Database (Denmark)

    Keilow, Maria; Friis-Hansen, Mette; Henze-Pedersen, Sofie

    udgangspunkt i det pædagogiske selvevalueringsredskab MinMestring. Begge indsatser har særligt fokus på elever med ADHD og ADHD-lignende vanskeligheder, men inddrager alle elever. Evalueringen viser overordnet, at indsatserne forbedrer elevernes koncentrationsevner, men kun i begrænset omfang gavner deres...

  20. Selskabsstyring i unoterede virksomheder

    DEFF Research Database (Denmark)

    Bennedsen, Morten

    2004-01-01

    I provide a short survey on recent research on the governance of closely held corporations. I focus on the strategic choice of ownership structure, the creation of family firms and the role of the board in the closely held corporation. Attention is payed to policyimplications of theresearch results....

  1. Kemikalier i tekstiler

    DEFF Research Database (Denmark)

    Larsen, Henrik Fred; Helweg, Christian; Rathmann Pedersen, Anne

    Rapporten indeholder en kortlægning af hvilke kemiske stoffer, der muligvis kan forekomme i færdige tekstiler og en screening af deres miljø- og sundhedsfarlighed. 22 stykker tekstiler på det danske marked er blevet analyseret i et laboratorium for de kemiske stoffer der vurderes udfra screeninge...

  2. Historie i konkurrencestaten

    DEFF Research Database (Denmark)

    Nielsen, Carsten Tage

    2015-01-01

    Artikelen sætter fokus på historiefaget i den danske grundskole set i konkurrencestatens perspektiv. Artiklen undersøger og diskuterer, hvordan konkurrencestatsdiskursen relaterer sig til den stående historiedidaktiske diskussion om historiebevidsthed (dannelse og identitet) og historisk tænkning...

  3. Lesson study i Danmark?

    DEFF Research Database (Denmark)

    Mogensen, Arne

    2009-01-01

    Der beskrives et japansk lesson study forløb, og det diskuteres i hvilket omfang, de gode japanske erfaringer kan overføres til dansk matematikundervisning.......Der beskrives et japansk lesson study forløb, og det diskuteres i hvilket omfang, de gode japanske erfaringer kan overføres til dansk matematikundervisning....

  4. Etniske minoritetsunge i Danmark

    DEFF Research Database (Denmark)

    Liversage, Anika; Christensen, Christiane Præstgaard

    Denne rapport tegner et bredt billede af 18-årige etniske minoritetsunges liv i Danmark i dag, sammenlignet med jævnaldrende fra den danske majoritet. Rapporten kommer rundt om en lang række emner som familieforhold og - ressourcer, uddannelse, helbred og trivsel, venner og fritidsliv, stoffer, a...

  5. God stil i professionsopgaver

    DEFF Research Database (Denmark)

    Petersen, Kit Stender; Christoffersen, Ditte Dalum

    "God stil i professionsopgaver" er en vejledende bog, der sikrer dig en god arbejdsproces og det bedst mulige resultat. Du får hjælp fra start til slut, og information om alt fra hvordan du stiller et videnskabelige spørgsmål til hvordan du laver en analyse og sikrer korrekt formalia. "God stil i...

  6. Individuel gasopvarmning i etageboliger

    DEFF Research Database (Denmark)

    Kjelstrup, S.; Nielsen, V.; Ovesen, K.

    Meddelelsen beskriver et kombineret udeluft- og aftrækssystem, der ligesom en skorsten føres gennem bygningens etager op over tag. Systemet giver mulighed for tilslutning af gaskedler i de enkelte lejligheder. Der skitseres et dansk udviklet system, og det vises hvordan det kan anvendes både i ny...

  7. Danske erfaringer i Afghanistan

    DEFF Research Database (Denmark)

    Nielsen, Thomas Galasz

    2016-01-01

    Der er høstet mange forskellige danske erfaringer i Afghanistan gennem det civile og militære engagement fra 2001 til 2014, men det er muligt at samle dem i tre områder, hvor der er mulighed for at forbedre fremtidige indsatser: Prioritet til integrerede indsatser, lokal sikkerhedsskabelse og...

  8. Westerns i vildrede

    DEFF Research Database (Denmark)

    Bjerre, Thomas Ærvold

    2013-01-01

    Disney's "The Lone Ranger" er seneste bevis på, at westernfilmen stadig lever i Hollywood. Men hvor genren engang dominerede og satte dagsordenen, lever den i dag et mere risikabelt liv spændt ud mellem uslyldig underholdning og seriøs samfundsdebat...

  9. I M L Das

    Indian Academy of Sciences (India)

    Home; Journals; Bulletin of Materials Science. I M L Das. Articles written in Bulletin of Materials Science. Volume 33 Issue 4 August 2010 pp 383-390 Electrical Properties. Temperature dependence of electromechanical properties of PLZT /57/43 ceramics · A K Shukla V K Agrawal I M L Das Janardan Singh S L Srivastava.

  10. Egoizam i altruizam

    OpenAIRE

    Sadžakov, Slobodan

    2015-01-01

    U radu se tematiziraju problemi egoizma i altruizma, prije svega u kontekstu njihova etičkoga razmatranja. U okviru teksta nastoje se promisliti veze ovih dvaju fenomena s pojmovima moralne individualnosti, moralne dužnosti, modernog identiteta i strukture praktičkoga »svijeta«.

  11. Aromater i drikkevand

    DEFF Research Database (Denmark)

    Nyeland, B. A.; Hansen, A. B.

    DMU har den 10. Juni 1997 afholdt en præstationsprøvning: Aromater i drikkevand. Der deltog 21 laboratorier i præstationsprøvningen. Prøvningen omfattede 6 vandige prøver og 6 ampuller indeholdende 6 aromater. Laboratorierne spikede de tilsendte vandprøver med indholdet fra ampullerne...

  12. Nuclear physics I

    International Nuclear Information System (INIS)

    Elze, T.

    1988-01-01

    This script consisting of two parts contains the matter of the courses Nuclear Physics I and II, as they were presented in the winter term 1987/88 and summer term 1988 for students of physics at Frankfurt University. In the present part I the matter of the winter term is summarized. (orig.) [de

  13. Libanon i regionen

    DEFF Research Database (Denmark)

    Schmidt, Søren

    2011-01-01

    Artiklen behandler Libanons regionale relationer, herunder forholdet til Syrien og Iran. Syrien har gjort krav på Libanon, grebet ind i borgerkrigten, rømmet landet, bevaret agenter og en vis opbakning, er mistænkt i Hariri-sagen, og er senest dybt påvirket af det arabiske forår. Ikke en nem, men...

  14. Storlandbrug i Danmark

    DEFF Research Database (Denmark)

    Rentz-Petersen, Nee

    2011-01-01

    Med udgangspunkt i storlandbrugets udfordringer med at indpasse deres nye produktionsanlæg i det danske kulturlandskab, introduceres et nyt princip for helhedsplanlægning for nye storlandbrug anlagt på 'bar mark'. Forslaget indeholder produktionsbygning med tilhørende tekniske anlæg, samt 3 bepla...

  15. Tintin i Sovjet

    DEFF Research Database (Denmark)

    Eskildsen, Mads Büchert

    2014-01-01

    Trods det næsten umulige i at fremskaffe objektiv information om, hvad der foregik i Sovjetunionen, tegnede og skrev Hergé et helt album om et land, et folk og et system, han ikke havde meget tilovers for. Det interessante er, at hans ukritiske tilgang til kritikken ramte plet på mange punkter, der...

  16. iPhone Idol

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Technophiles around the world are snapping up Apple’s new iPhone. How much longer must the Chinese wait?The iPhone, a faster and more versatile version of Apple Inc.’s original touch-based mobile handset, has been a market hit since it went on

  17. EU ligger i Danmark!

    DEFF Research Database (Denmark)

    Adler-Nissen, Rebecca

    2007-01-01

    EU må ikke reduceres til et spørgsmål om folkeafstemninger. Den virkelige udfordring handler om, hvordan EU-beslutninger om alt fra dyretransporter til terrorbekæmpelse kan kontrolleres på en demokratisk måde. De beslutninger, der bliver truffet i Bruxelles er i højeste grad en del af den nationa...

  18. Fast ejendom, I

    DEFF Research Database (Denmark)

    Munk-Hansen, Carsten

    , værdiforringende forhold, der ligger uden for ejendommen og andre særlige tilfælde. Bogen uddyber andre emner omtalt i 1. udgave, eksempelvis erhvervelsesbetingelser, købsoptioner, ansvarsfraskrivelse, licitationssalg mv. Bogen er det første af tre planlagte bind om fast ejendom: I Overdragelsen, II Bolighandlen...

  19. Kystnatur i klimaklemme: Fuglelivet

    DEFF Research Database (Denmark)

    Clausen, Kevin Kuhlmann

    Fra fugleperspektiv er de danske kysthabitater unikke i international sammenhæng, og vores lavvandede områder, mudderflader og strandenge udgør tilsammen en habitatmosaik, som er hjemsted for tusindvis af ynglende og trækkende fugle. Udbredelsen af disse levesteder beror på fysiske egenskaber i g...

  20. Wii i Trige

    DEFF Research Database (Denmark)

    Aarhus, Rikke; Cedergren, Anni; Grönvall, Erik

    2010-01-01

    Projekt Wii-i-Trige (feb.-juli 2009) blev igangsat i forbindelse med Konsortiet for Brugerdreven Sundhedsinnovations arbejde med ældre og teknologi. Undersøgelsen har haft fokus på ældres anvendelse af Nintendo Wii, samt på hvorvidt computerspil med fysisk interaktion kan motivere og bidrage til ...

  1. Forskellighed i supervision

    DEFF Research Database (Denmark)

    Petersen, Birgitte; Beck, Emma

    2009-01-01

    Indtryk og tendenser fra den anden danske konference om supervision, som blev holdt på Københavns Universitet i oktober 2008......Indtryk og tendenser fra den anden danske konference om supervision, som blev holdt på Københavns Universitet i oktober 2008...

  2. Symposium i Anvendt Statistik

    DEFF Research Database (Denmark)

    resultater. Symposiet er denne gang inddelt i følgende sessioner: Arbejdsmarkedsøkonometri, Market Research I-II, Industriel Statistik, Statistiske Metoder, Surveys og Internetundersøgelser, Demografi og Finan-siel økonometri. Med symposiet tilstræbes det at skabe et forum for tværfaglig inspiration og...

  3. Biodiversitet i dyrket skov

    DEFF Research Database (Denmark)

    Petersen, Flemming Rune

    . Rapportens anden del er en gennemgang af fire væsentlige indgreb, der påføres skovøkosystemerne ved dyrkningspraksis. Rapportens tredje del behandler særlige driftstyper, der fremmer et stort naturindhold i danske skove, med udgangspunkt i Naturskovsstrategiens beskrivelse af "gamle driftsformer"....

  4. At vandre i flok

    DEFF Research Database (Denmark)

    Fibiger, Marianne Qvortrup

    2017-01-01

    En artikel om moderne hinduisme i Mauritius med fokus på processioner, men også med en kritisk tilgang til begrebet levende religion.......En artikel om moderne hinduisme i Mauritius med fokus på processioner, men også med en kritisk tilgang til begrebet levende religion....

  5. Narrativ vejledning i skolen

    DEFF Research Database (Denmark)

    Plant, Peter

    2009-01-01

    Anmeldelse af John M. Winslade og Gerald D. Monk (2008). Narrativ vejledning i skolen. Virum: Dansk Psykologisk Forlag   Udgivelsesdato: FEB......Anmeldelse af John M. Winslade og Gerald D. Monk (2008). Narrativ vejledning i skolen. Virum: Dansk Psykologisk Forlag   Udgivelsesdato: FEB...

  6. Mening i arbejdslivet

    DEFF Research Database (Denmark)

    Ravn, Ib

    2008-01-01

    begrebet, hvorfor det defineres her: Et fænomen giver mening, når man indser, hvordan det indgår i en større sammenhæng. Mening i arbejdslivet gives da af oplevelsen af, at man gennem sit arbejde indgår i en større sammenhæng. En konceptualisering med fire delfaktorer udledes, der tilsammen skønnes at give...... mening i arbejdslivet: 1. Styrkerealisering: Den enkelte bruger sine styrker og talenter; 2. Værdiskabelse: Ens arbejdsplads skaber reel værdi og livskvalitet for kunder og brugere. 3. Bidrag: Man oplever at yde et vigtigt bidrag gennem sit arbejde. 4. Fællesskab: Man indgår i et produktivt fællesskab...

  7. Rummelighed i praksis

    DEFF Research Database (Denmark)

    Holt, Helle; Mehlsen, Line

    ansættelser af personer i fleksjob – skal etableres på en måde, så det kommer både arbejdspladserne og de inkluderede til gode. Undersøgelsen er baseret på dybdegående interview i tre kommuner og på ni arbejdspladser. Der er udført interview med direktioner, HR-afdelinger, jobcentermedarbejdere......, tillidsrepræsentanter, lokale ledere, kommunalt ansatte samt personer, der har været i fx virksomhedspraktik, i et løntilskudsforløb, eller som er blevet ansat i et fleksjob på en kommunal arbejdsplads. Undersøgelsen er igangsat og finansieret af Kommunernes Landsforening og Forhandlingsfælleskabet....

  8. Engineering i skolen

    DEFF Research Database (Denmark)

    Auner, Suzie; Daugbjerg, Peer; Nielsen, Keld

    Denne didaktik er udarbejdet i forbindelse med projektet “Engineering i skolen” (EiS), som har til formål at bringe engineering ind i naturfagsundervisningen i grundskolen. EiS er et samarbejde mellem Engineer the future, VIA University College, Astra og Naturvidenskabernes Hus, UCC samt lærere fra...... grundskolen ligeledes kan have glæde af at læse med. Didaktikken er blevet til på grundlag af en række forarbejder. Et indledende litteraturstudium dannede udgangspunkt for at opstille nogle principper for en engineering-didaktik, der kan fungere i dansk grundskolekontekst. Sideløbende blev der gennemført en...

  9. Feedback i matematik

    DEFF Research Database (Denmark)

    Sortkær, Bent

    2017-01-01

    Feedback bliver i litteraturen igen og igen fremhævet som et af de mest effektive midler til at fremme elevers præstationer i skolen (Hartberg, Dobson, & Gran, 2012; Hattie & Timperley, 2007; Wiliam, 2015). Dette på trods af, at flere forskere påpeger, at feedback ikke altid er læringsfremmende...... (Hattie & Gan, 2011), og nogle endda viser, at feedback kan have en negativ virkning i forhold til præstationer (Kluger & DeNisi, 1996). Artiklen vil undersøge disse tilsyneladende modstridende resultater ved at stille spørgsmålet: Under hvilke forudsætninger virker feedback i matematik læringsfremmende......? Dette gøres ved at dykke ned i forskningslitteraturen omhandlende feedback ud fra en række temaer for på den måde at besvare ovenstående spørgsmål....

  10. I Play Roles, Therefore I Am

    DEFF Research Database (Denmark)

    Sandvik, Kjetil; Waade, Anne Marit

    2007-01-01

    -reflecting activity is getting an increasingly growing attention from the media, and the fact that educators, teachers, and development consultants have discovered the potential inherent in role-playing, both as an idea and a strategy. In this essay, we place leisure role-playing in a broader perspective, not only...... Rollespil - i æstetisk, pædagogisk og kulturel sammenhæng (2006), which is the first Danish academic anthology on role-playing as an aesthetic, educational, and cultural phenomenon....

  11. Radiological assistance program: Region I. Part I

    International Nuclear Information System (INIS)

    Musolino, S.V.; Kuehner, A.V.; Hull, A.P.

    1985-01-01

    The purpose of the Radiological Assistance Program (RAP) is to make DOE resources available and provide emergency assistance to state and local agencies in order to control radiological hazards, protect the public health and safety, and minimize the loss of property. This plan is an integral part of a nationwide program of radiological assistance established by the US DOE, and is implemented on a regional basis. The Brookhaven Area Office (BHO) Radiological Assistance Program is applicable to DOE Region I, which consists of the New England States, New York, New Jersey, Pennsylvania, Delaware, Maryland and the District of Columbia. The BHO RAP-1 has been developed to: (a) ensure the availability of an effective radiological assistance capability to ensure the protection of persons and property; (b) provide guidelines to RAP-1 Team personnel for the evaluation of radiological incidents and implementation of corrective actions; (c) maintain liaison with other DOE installations, Federal, State and local organizations which may become involved in radiological assistance operations in Region I; and (d) encourage development of a local capability to cope with radiological incidents

  12. Orthopaedic management of Hurler's disease after hematopoietic stem cell transplantation : A systematic review

    NARCIS (Netherlands)

    van der Linden, Marleen H.; Kruyt, Moyo C.; Sakkers, Ralph J. B.; de Koning, Tom J.; Oner, F. Cumhur; Castelein, Rene M.

    The introduction of hematopoietic stem cell transplantation (HSCT) has significantly improved the life-span of Hurler patients (mucopolysaccharidosis type I-H, MPS I-H). Yet, the musculoskeletal manifestations seem largely unresponsive to HSCT. In order to facilitate evidence based management, the

  13. <i>Femeniasia, novus genus Carduearumi>

    Directory of Open Access Journals (Sweden)

    Susanna de la Serna, Alfonso

    1988-01-01

    Full Text Available The systematic position of <i>Centaurea balearicai> Rodríguez Femenias (<i>Asteraceae-Cardueae> is studied. A detailed analysis or its anatomy, with special reference to the carpology, forces the author lo reject its inclusion in the genus <i>Centaurea> or in any other genus or the tribe. A new genus, <i>Femeniasia> Susanna. is hence described, and the new nomenclatural combinari en <i>Femeniasia balearicai> (Rodríguez Femenías Susanna is proposed. The relationship or <i>Femeniasia> and the other genera of the tribe is discussed; the author concludes that <i>Femeniasia> is a quite isolated genus in the <i>Cardueae-Carduinae> .

    [ca] Estudi de la posició sistemàtica de <i>Centaurea balearicai
    > Rodríguez Femenías (<i>Asteraceae-Cardueae> . De l'anàlisi minuciosa de la seva anatomia, especialment de les seves cípseles, resulta que l'esmentada espècie no és cap <i>Centaurea>; tampoc pot ésser inclosa en cap del altres gèneres de la tribu. En conseqüència, hom descriu un nou gènere, <i>Femeniasia> Susanna, i proposa la combinació <i>Femeniasia balearicai> (Rodríguez Femenías Susanna. Son analitzades les possibles relacions de parentiu amb d'altres gèneres del grup i l'autor arriba a la conclusió que <i>Femeniasia> és un gènere clarament isolat entre les <i>Cardueae-Carduinae>.

  14. Naturlig ventilation i enfamiliehuse

    DEFF Research Database (Denmark)

    Bergsøe, N.C.

    Meddelelsen beskriver resultaterne af en række beregninger foretaget ved anvendelse af et computerprogram. Beregningerne har til formål at belyse forskellige parametres indvirkning på funktionen af et naturligt ventilationssystem. Blandt andet belyses systemets afhængighed af aftrækskanalernes di...... dimension, udeluftventilarealet og placeringen af aftrækskanalernes udmunding i tagfladen. Derudover gengives i kortfattet form de væsentligste konklusioner af udvalgte publikationer, som behandler særlige forhold vedrørende naturlig ventilation i praksis....

  15. iTunes music

    CERN Document Server

    Katz, Bob

    2013-01-01

    Apple's exciting new Mastered for iTunes (MFiT) initiative, introduced in early 2012, introduces new possibilities for delivering high-quality audio. For the first time, record labels and program producers are encouraged to deliver audio materials to iTunes in a high resolution format, which can produce better-sounding masters. In iTunes Music, author and world-class mastering engineer Bob Katz starts out with the basics, surveys the recent past, and brings you quickly up to the present-where the current state of digital audio is bleak. Katz explains the evolution of

  16. Giochiamo con i robot

    Directory of Open Access Journals (Sweden)

    Andrea Bonarini

    2009-01-01

    Full Text Available "Giochiamo con i robot" e' un laboratorio interattivo per grandi e piccini realizzato per l'edizione 2007 del Festival della Scienza di Genova. Lungo un percorso che va dalla telerobotica alla robotica evolutiva, il laboratorio sviluppa il tema di dare intelligenza ai robot. Questo percorso, le cui tappe sono le varie installazioni, si conclude nella "bottega" dove e' possibile costruire e programmare i propri robot o smontare e modificare quelli esposti durante il percorso didattico. I visitatori sono coinvolti in attivita' ludiche grazie alle quali possonoentrare in contatto con alcune delle idee potenti della robotica,

  17. Loyalitet i praksis

    DEFF Research Database (Denmark)

    Breddam, Mads Dupont

    Loyalitet i praksis – en etnologisk kulturanalyse af fænomenet den loyale forbruger , er en kvalitativ kulturanalyse af, hvordan loyalitet skabes i forbindelse med den danske detailhandels - og andelsorganisation Coop og Coops loyalitetsprogram CoopPlus. Undersøgelserne bygger på et empirisk...... undersøger , hvordan loyalitet gøres i praksis, ved at indkredse det analytiske og multiple fænomen den loyale forbruger . Tilgangen er, at loyalitet praktiseres, nå r medarbejdernes, medlemmernes og medejernes versioner af den loyale forbruger relateres til hinanden. Med andre ord fokuserer forfatteren på...

  18. Integreret rapportering i praksis

    DEFF Research Database (Denmark)

    Pontoppidan, Caroline Aggestam; Sonnerfeldt, Amanda

    2017-01-01

    Formålet med denne artikel er at bidrage til en dybere forståelse af fænomenet integreret rapporte-ring () og, hvordan IIRC’s begrebsramme afspejles i praksis i virksomheder og af interessen-ter. Artiklen tager udgangspunkt i nyere empiriske forskningsprojekter, som bringer viden omkring......; 1) brugeres (stakeholders) informationsbehov, herunder anvendelsen og nytteværdien af og 2) praktiske udfordringer samt ’best practice’ tendenser inden for (ACCA, 2016; ACCA 2017; AcE, 2017; IFAC 2017)....

  19. Joves i estructura social

    OpenAIRE

    Merino Pareja, Rafael

    2008-01-01

    Ubicar els joves en l’estructura social no és un tema fàcil. Bàsicament tenim dos tipus de problemes, un conceptual i un altre metodològic. Tenim problemes per definir el concepte de joventut, i el canvi social d’una societat industrial relativament estable a una societat postindustrial, del coneixement, del risc, informacional o com se li vulgui dir, ha agreujat considerablement els esforços teòrics per entendre el jovent. Tenim problemes per mesurar, per operativitzar els conceptes. Tot i q...

  20. iAnn

    DEFF Research Database (Denmark)

    Jimenez, Rafael C; Albar, Juan P; Bhak, Jong

    2013-01-01

    We present iAnn, an open source community-driven platform for dissemination of life science events, such as courses, conferences and workshops. iAnn allows automatic visualisation and integration of customised event reports. A central repository lies at the core of the platform: curators add...... submitted events, and these are subsequently accessed via web services. Thus, once an iAnn widget is incorporated into a website, it permanently shows timely relevant information as if it were native to the remote site. At the same time, announcements submitted to the repository are automatically...

  1. Genetic and other diseases in the pottery of Tumaco-La Tolita culture in Colombia-Ecuador.

    Science.gov (United States)

    Bernal, J E; Briceno, I

    2006-09-01

    The people of Tumaco-La Tolita culture inhabited the borders of present-day Colombia and Ecuador. Already extinct by the time of the Spaniards arrival, they left a huge collection of pottery artifacts depicting everyday life; among these, disease representations were frequently crafted. In this article, we present the results of the personal examination of the largest collections of Tumaco-La Tolita pottery in Colombia and Ecuador; cases of Down syndrome, achondroplasia, mucopolysaccharidosis I H, mucopolysaccharidosis IV, a tumor of the face and a benign tumor in an old woman were found. We believe these to be among the earliest artistic representations of disease.

  2. Flip i cellemembranen

    DEFF Research Database (Denmark)

    Vestergaard, Anna Lindeløv; Vilsen, Bente; Andersen, Jens Peter

    2012-01-01

    Forskellen på fordelingen af forskellige fedtmolekyler i cellemembranens ydre og indre lag er afgørende for membranens funktion. Ny forskning kaster lys over, hvordan denne asymmetri opretholdes....

  3. I-15 North Project

    Science.gov (United States)

    2013-11-05

    Goals of this project were as follows: (1) Conduct a comprehensive evaluation study on Nevada's I-15 North Design Build Project; (2) Analyze project implementation with respect to construction zone rules by which the contractor had to abide; (3) Anal...

  4. EPA iComplaints

    Data.gov (United States)

    U.S. Environmental Protection Agency — The iComplaints system is an enterprise-level COTS (Commercial Off-The-Shelf) product that provides all of the funtionality required to collect, track, manage,...

  5. Handlekompetence i naturfag

    DEFF Research Database (Denmark)

    Andersen, Thomas Bech Lillelund

    2012-01-01

    is to be seen as future potentials driven by will, but also because of the action part. I have divided knowledge in Aristotle’s three types of knowledge: episteme, techne and phronesis and argues that competences, contains all three kinds of knowledge. I argue that most assessment is on epistemic knowledge only...... to assess action competence. Inquiry suits well socio cultural theories of learning and competence goals, partly because of action and problem solving. Giving 8 grade class in science a new and authentic problem to solve I have tried to see signs of all three kinds of knowledge to estimate if action...... in Aristotle’s three types of knowledge: episteme, techne and phronesis and argues that competences, contains all three kinds of knowledge. I argue that most assessment is on epistemic knowledge only, leaving techne and phronesis out of assessment. Goals not assessed tend to disappear on behalf of easily...

  6. Madspild i familier

    DEFF Research Database (Denmark)

    Winkel, Thomas Dyrmann

    særligt henblik på forholdet mellem familiens sociale dynamikker, praksisser og hverdagens organisering. Et misforhold mellem hverdagens organisering og familiemedlemmernes madpraksisser er en væsentlig årsag til madspild. Teorien, der forklarer, hvorfor familiers praksis forårsager madspild, ligger til...... forskningsspørgsmål, forskningsdesign analyse og konklusioner. I afhandlingen omhandler et studie af familiers praksis, der forårsager madspild. Resultatet er en teori herom og en metode til at reducere madspildet gennem ændring af familiers praksisser. Jeg undersøger i familiestudiet seks børnefamiliers praksis med...... grund for en metode til at reducere madspildet. Metoden bygger primært på praksisteori og tager højde for etiske implikationer i ændringen af familiers praksis. Den udviklede metode er tilpasset anvendelse i organisatorisk praksis og indeholder et konkret redskab til brug før, under og efter...

  7. Katedralskolen i Transformation

    DEFF Research Database (Denmark)

    2016-01-01

    stadig mulige byggefelter, hvis anvendelse kan ommøblere funktioner, så det imødekommer nye undervisningsformer og det sociale liv vi har i dag. En ny tilbygning står på skolens ønskeliste. Således er skolen i fortsat transformation. I tegninger og modeller fremviser udstillingen konkurrenceforslag til...... nye tilbygninger til Katedralskolen, der fortsætter traditionen om transformation og som fortsætter fortætning af skolen og dermed af byen. Byggeprogrammet er blevet diskuteret på baggrund af brugerinterviews af Katedralskolens lærere og elever. Arkitekter fra Arkitekttegnestuen Kjær & Richter i...

  8. iPSC Core

    Data.gov (United States)

    Federal Laboratory Consortium — The induced Pluripotent Stem Cells (iPSC) Core was created in 2011 to accelerate stem cell research in the NHLBI by providing investigators consultation, technical...

  9. Realismen i dansk film

    DEFF Research Database (Denmark)

    Langkjær, Birger

    Realisme som begreb og filmpraksis (Hvad er realisme? Forestillinger om realisme og realismen i praksis; Realismens niveauer og elementer; Realismen som filmpraksis: mellem genrefilm og artfilm); Skæbne og tilfældighed...

  10. Mellemledelse i forbedringskultur

    DEFF Research Database (Denmark)

    Hansen, David; Jørgensen, Rasmus

    2013-01-01

    Lean er kommet for at blive. I over et årti har produktions- og serviceledelse i hele verden taget Toyotas produktionssystem til sig for at øge effektivitet og skabe løbende forbedringer. Japanske termer som kanban, muda og kaizen er ved at være mainstream sammen med forståelsen for floweffektivi......Lean er kommet for at blive. I over et årti har produktions- og serviceledelse i hele verden taget Toyotas produktionssystem til sig for at øge effektivitet og skabe løbende forbedringer. Japanske termer som kanban, muda og kaizen er ved at være mainstream sammen med forståelsen...

  11. Can I help you?

    DEFF Research Database (Denmark)

    Andersen, Bente Thorup; Lund, Marianne

    2010-01-01

    At servicere udenlandske studerende er blevet hverdag på mange uddannelsesbiblioteker. Uddannelsesbibliotekaren har set på, hvordan de udenlandske studerende er, og på hvordan bibliotekerne kan komme dem i møde....

  12. Romerske veje i Syditalien

    DEFF Research Database (Denmark)

    Bekker-Nielsen, Tønnes

    2018-01-01

    I andet århundrede e.Kr. lod kejser Trajan en ny, 300 kilometer lang hovedvej anlægge til aflastning af en eksisterende vej gennem Syditalien. Ved at sammenligne de to vejanlæg får man et enestående indblik i den romerske ingeniørkunsts udvikling over en periode på 400 år.......I andet århundrede e.Kr. lod kejser Trajan en ny, 300 kilometer lang hovedvej anlægge til aflastning af en eksisterende vej gennem Syditalien. Ved at sammenligne de to vejanlæg får man et enestående indblik i den romerske ingeniørkunsts udvikling over en periode på 400 år....

  13. I-centric Communications

    CERN Document Server

    Arbanowski, S; Steglich, S; Popescu-Zeletin, R

    2001-01-01

    During the last years, a variety of concepts for service integration and corresponding systems have gained momentum. On the one hand, they aim for the interworking and integration of classical telecommunications and data communications services. On the other hand, they are focusing on universal service access from a variety of end user systems. Looking at humans' communication behavior and communication space, it is obvious that human beings interact frequently in a set of contexts in their environment (communication space). Following this view, we want to build communication systems on the analysis of the individual communication spaces. The results are communication systems adapted to the specific demands of each individual. The authors introduce I-centric Communication Systems, an approach to design communication systems which adapt to the individual communication space and individual environment and situation. In this context "I" means I, or individual, "Centric" means adaptable to I requirements and a ce...

  14. Test i folkeskolen

    DEFF Research Database (Denmark)

    I bogen defineres og afgrænses testbegrebet, og det påvises, hvad test kan, og hvad de ikke kan. Desuden gennemgår og diskuterer bogens forfattere, hvordan skolen påvirkes af testkulturen, men også hvordan skolens lærere kan anvende testning og testresultater i deres undervisning, så den pædagogi......I bogen defineres og afgrænses testbegrebet, og det påvises, hvad test kan, og hvad de ikke kan. Desuden gennemgår og diskuterer bogens forfattere, hvordan skolen påvirkes af testkulturen, men også hvordan skolens lærere kan anvende testning og testresultater i deres undervisning, så den...

  15. I-94 Automation FAQs

    Data.gov (United States)

    Department of Homeland Security — In order to increase efficiency, reduce operating costs and streamline the admissions process, U.S. Customs and Border Protection has automated Form I-94 at air and...

  16. Meteorologia i clima

    OpenAIRE

    Alarcón Jordán, Marta; Casas Castillo, M. del Carmen

    1999-01-01

    Aquest llibre constitueix una introducció a l'estudi de l'atmosfera en els camps de la meteorologia i la climatologia. Ha estat concebut especialment com a eina de suport per als estudiants de ciències i tecnologia que inicien l'estudi d'aquests temes. El llibre incorpora un programa informàtic de simulació d'un canvi climàtic. S'hi exposen les característiques generals de l'atmosfera, la seva estructura física i la composició química i també es fa una introducció al problema de la contaminac...

  17. I Remember Highlander

    Science.gov (United States)

    Williams-Hawkins, Maria

    2016-01-01

    "I Remember Highlander" reflects on the life choices of Marion Barry and Herman Henning Jr., fraternity brothers who sought the same goal but took different paths. The essay examines cultural and family situations that shaped lives and decisions.

  18. I epidemisk alarmtilstand

    DEFF Research Database (Denmark)

    Østergaard, Lise Rosendal

    2015-01-01

    Sundhedsarbejdere i Burkina Faso har den svære opgave at arbejde under materielle betingelser præget af knaphed. Ebola tydeliggør, hvad den forskel betyder i liv. Der har været en del opmærksomhed på risikoen for, at befolkningens tillid til sundhedssystemet forringes. Vi bør også tænke på risikoen...

  19. Sang i Demensplejen

    DEFF Research Database (Denmark)

    Ridder, Hanne Mette Ochsner

    2004-01-01

    For mange svært demensramte er det umuligt at deltage i en aktivitet med andre personer. De har svært ved at forstå, hvad der foregår omkring dem, og bliver urolige og "forstyrrende". Eller de falder i søvn. Artiklen giver et bud på, hvordan den gruppe af demensramte, som ofte beskrives som uroli...

  20. Ulighed i sundhed

    DEFF Research Database (Denmark)

    Diderichsen, Finn

    2016-01-01

    Ulighed i sundhed er af Bo Könberg blevet løftet frem som et særligt nordisk samarbejdsområde. Den nordiske velfærdsstat står og falder med en høj beskæftigelse, men ulighed i sundhed handler om en alt stærkere kobling mel-lem kort uddannelse, ingen eller usikker beskæftigelse og dårligt helbred...

  1. Kompendium i generelle analyseprincipper

    DEFF Research Database (Denmark)

    Petersen, Mette la Cour

    Introduktion til arbejdet i det kliniske laboratorie. Betydende cifre og videnskabelig angivelse af tal. Vandkvaliteter i laboratoriet. Volumetrisk udstyr og vejeudstyr. Volumetrisk udstyr - tællekammer. Fortyndinger. Kvalitetssikring. Antikoagulans. Hygiejne for bioanalytikere. Sedimentation og...... centrifugering. Kromatografi. Elektroforese. Plasmaproteiner og deres diagnostiske betydning. Blodtypebestemmelse ved søjleagglutination. Fældning og dialysering af proteiner. Flowcytometri. Massespektrometri. Filtrering. Lyslære. Lysfeltmikroskopi. Fotometri (absorbtionsfotometri). Andre fotometriske metoder...

  2. Paris i kroppen

    DEFF Research Database (Denmark)

    Laursen, Bjørn

    2003-01-01

    fodtøj ud til læserne, så de kan trave rundt sammen med dens forfatter i ”Byernes By”. Det er omdrejningspunktet for artiklen, at vi er multisensoriske individer som ser og oplever hele livet i-med-gennem kroppen. Ifølge Laursen er vi paradoksalt nok blevet så ekvilibristiske til det at vi også er...

  3. T I Eldho

    Indian Academy of Sciences (India)

    Home; Journals; Sadhana. T I Eldho. Articles written in Sadhana. Volume 31 Issue 6 December 2006 pp 743-754. Hydrodynamic modelling of flow over a spillway using a two-dimensional finite volume-based numerical model · M R Bhajantri T I Eldho P B Deolalikar · More Details Abstract Fulltext PDF. Spillway flow, a ...

  4. Deltagelse i arbejdslivet

    DEFF Research Database (Denmark)

    Sylvestersen, Janna Bohn; Sevel, Claus

    2012-01-01

    Med fokus på det lønnede arbejdsmarked anvendes teori om Virksomhedens sociale kapital, Occupational justice og Stigma til at forstå og eksemplificere problematikker i den erhvervsrettede rehabilitering......Med fokus på det lønnede arbejdsmarked anvendes teori om Virksomhedens sociale kapital, Occupational justice og Stigma til at forstå og eksemplificere problematikker i den erhvervsrettede rehabilitering...

  5. Taler i krisens tegn

    DEFF Research Database (Denmark)

    Hansen, Jette Barnholdt

    2012-01-01

    I dette indlæg anmelder jeg Helle Thorning-Schmidts og Dronning Margrethe II's nytårstaler med særligt fokus på deres mundtlige fremførelse.......I dette indlæg anmelder jeg Helle Thorning-Schmidts og Dronning Margrethe II's nytårstaler med særligt fokus på deres mundtlige fremførelse....

  6. Dagslysberegninger i praksis

    DEFF Research Database (Denmark)

    Iversen, Anne; Roy, Nicolas; Hvass, Mette

    Formålet med rapporten er at opnå en bedre forståelse af, hvad en dagslysberegning viser, og hvordan de forskellige beregningsprogrammer beregner i forhold til hinanden. Erfaringer har vist, at beregningsresultaterne fra to beregningsprogrammer til dagslysberegning kan være meget forskellige i de...... simuleringsprogrammer er undersøgt og sammenlignet. Det drejer sig om: Radiance, Daysim, VELUX Daylight Visualizer, Dialux, Ecotect, Ecotect / Radiance, IESve, LightCalc, Relux Radiosity og Relux Raytracing....

  7. Unges motivation i udskolingen

    DEFF Research Database (Denmark)

    Pless, Mette; Katznelson, Noemi; Hjort-Madsen, Peder

    Om hvordan de unge i udskolingen skaber lyst og motivation for læring. Med afsnit om hvad motivation er, hvordan den fremmes hos unge og kombineres med et liv udenfor skolen......Om hvordan de unge i udskolingen skaber lyst og motivation for læring. Med afsnit om hvad motivation er, hvordan den fremmes hos unge og kombineres med et liv udenfor skolen...

  8. Motivation i erhvervsuddannelserne

    DEFF Research Database (Denmark)

    Katznelson, Noemi; Brown, Rikke

    Denne rapport handler om de mange unge, der befolker erhvervsuddannelserne i Danmark. Den sætter fokus på, hvad der motiverer disse unge til at være på uddannelserne og på den hverdag, de har på skolerne. Rapporten giver et indblik i, hvordan de unge oplever mødet med faget, pædagogikken og lærer...

  9. Ventilation i industrien

    DEFF Research Database (Denmark)

    Valbjørn, O.

    I en række afsnit belyses problemer med træk, kulde, varme, og luftforurening på industriens arbejdspladser, og hvordan man ved ventilation og bygningsudformning kan bekæmpe disse gener. Hvert afsnit kan i princippet læses for sig, og anvisningen kan derfor bruges som håndbog, både af de der er...

  10. Kompetanse i styrene

    OpenAIRE

    Andersen, Kjersti

    2013-01-01

    Executive Master of Management fra Handelshøyskolen BI, 2013 Styrekompetanse i bygg- og eiendomsbedrifter er tema for denne oppgaven. Aksjeloven krever at bedriften skal ha et styre, men sier lite eller ingenting om hvordan styret skal arbeide ei heller hvilken kompetanse som er nødvendig for at styret skal fungere best mulig. Hvilke refleksjoner eierne og styremedlemmene har gjort seg om egen og styrets kompetanse, sammensetning, livssyklus og styrevalg blir analysert i oppgaven. Fo...

  11. Foregribende felttog i Mesopotamien

    DEFF Research Database (Denmark)

    Ousager, Asger

    2005-01-01

    Dio antydede, at strid om passende styreform kan være tilstrækkeligt grundlag for et forebyggende angreb på en fremmed magt. Med fodfæste i Irak lægger USA nu pres på både Syrien og Iran. I sin tid brugte det romerske imperium Syrien som afsæt for angreb på den persiske storkonge, der herskede ov...

  12. Ni landsbyer i Danmark

    DEFF Research Database (Denmark)

    Larsen, Jacob Norvig

    Denne rapport beskriver en evaluering af statsstøttede forsøg med at styrke og udvikle mindre lokalsamfund. Forsøgene er gennemført i ni kommuner. Da der i disse år er stor opmærksomhed om de små byers og samfunds udviklingsmuligheder, har erfaringerne fra forsøgsprojekterne bred interesse. Forsø...

  13. Power as <i>Sumbolon>

    DEFF Research Database (Denmark)

    Dean, Mitchell

    2017-01-01

    himself used in his lectures during that decade. This figure and its “rule of halves” prevent us from reducing his analysis of power to one of liberal or economic government. This provides us with a framework for both how the international domain is approached in these investigations and how we might...... remain loyally unfaithful to his approach to the international and to power more broadly. I indicate three ways we might enact this ethos: by identifying a liberal international dispositif alongside Foucault’s diplomatic-military one associated with reason of state; by the comparative investigation...... the question of limits and their fecundity in reason of state and liberalism, in both domestic and international domains; and by viewing sovereignty not simply as grounded in a “right of death” but as the very condition of a biopolitics....

  14. I in generalized supergravity

    Energy Technology Data Exchange (ETDEWEB)

    Araujo, T.; O Colgain, E. [Asia Pacific Center for Theoretical Physics, Pohang (Korea, Republic of); Sakamoto, J.; Yoshida, K. [Kyoto University, Department of Physics, Kyoto (Japan); Sheikh-Jabbari, M.M. [Institute for Research in Fundamental Sciences (IPM), School of Physics, Tehran (Iran, Islamic Republic of)

    2017-11-15

    We showed in previous work that for homogeneous Yang-Baxter (YB) deformations of AdS{sub 5} x S{sup 5} the open string metric and coupling and as a result the closed string density e{sup -2Φ}√(g) remain undeformed. In this work, in addition to extending these results to the deformation associated with the modified CYBE or η-deformation, we identify the Page forms as the open string counterpart for RR fields and demonstrate case by case that the non-zero Page forms remain invariant under YB deformations. We give a physical meaning to the Killing vector I of generalized supergravity and show for all YB deformations: (1) I appears as a current for the center of mass motion on the worldvolume of a D-brane probing the background, (2) I is equal to the divergence of the noncommutativity parameter, (3) I exhibits ''holographic'' behavior where the radial component of I vanishes at the AdS boundary and (4) in pure spinor formalism I is related to a certain state in the BRST cohomology. (orig.)

  15. Why I Love Vendors

    Energy Technology Data Exchange (ETDEWEB)

    Wiley, H. S.

    2010-02-01

    In December, I attended the annual meeting of the American Society for Cell Biology, as I have done regularly for the last several decades. It is always a good way to catch up with old friends and look for the latest trends in cell biology. I rarely attend the talks, having found that they more reflect the fashion of the moment (or the past) than the direction of the field. Poster sessions are more to my liking, since they provide a chance to talk to enthusiastic young scientists in the trenches. But my favorite stop has always been the vendor booths. When I tell my friends that I love visiting vendor booths, most of them seem to think I am kidding. At meetings, many scientists seem to feel that vendors are necessary evils. They provide free candy and cheap pens in exchange for bombarding us with ads and scanning our badges. Like popup ads in web browsers, we have learned to both ignore and accept them as part of the landscape. It is unfortunate that we have become so inured to their presence. Talks and posters capture exciting research from the last few years or months, but vendor booths capture the future, offering one of the clearest visions of where a field is going.

  16. Samtale i stedet for vold

    DEFF Research Database (Denmark)

    Enevoldsen, Jørn Henrik; Drewniak, Henrik Thim

    Evaluering af den kriminalpræventive indsats: Samtale i steder for vold, der foregår i et samarbejde mellem skolerne i Esbjerg og Esbjerg politik under SSP-udvalget i Esbjerg.......Evaluering af den kriminalpræventive indsats: Samtale i steder for vold, der foregår i et samarbejde mellem skolerne i Esbjerg og Esbjerg politik under SSP-udvalget i Esbjerg....

  17. Forskning om tvang i misbrugsbehandling

    DEFF Research Database (Denmark)

    Deding, Mette

    I Danmark er der begrænset mulighed for brug af tvang i behandlingen af stof- og alkoholmisbrugere. Det kan kun finde sted i forbindelse med en kontrakt om ”frivillig tvang” i behandlingen af gravide alkoholmisbrugere. I Sverige og Norge giver loven mulighed for brug af tvang, og i USA er tvang l...

  18. Kontraktstyring i den kommunale sagsbehandling

    OpenAIRE

    Malmskov, Stine; Knudsen, Louise Leelo; Krogh, Ulrik Ullits; Taaensen, Rebekka Amos

    2007-01-01

    Sagsbehandlers motivation i forhold til sagsbehandling i det kommunale. Der bruges public choice, principal-agentteori samt motiationsteori i form af Frey, Herzberg og Maslow Sagsbehandlers motivation i forhold til sagsbehandling i det kommunale. Der bruges public choice, principal-agentteori samt motiationsteori i form af Frey, Herzberg og Maslow

  19. Neutral interstellar gas toward epsilon persei: H I, H2, D I, N I, O I

    International Nuclear Information System (INIS)

    Vidal, A.; Ferlet, R.; Laurent, C.; York, D.G.

    1982-01-01

    The study of the interstellar medium toward epsilon Per, a moderately reddened (E/sub B/-V = 0.1) B0.5 star, through the analysis of H I, D I, H 2 , N I, O I, and Ar I absorption features, revealed the following structure of the line of sight: (1) a main interstellar, cold (Tapprox.100 K; b = 2.9 km s -1 ) component, (2) a weak (approx.1% of the main one) probably cold component, and (3) a weak (also approx.1% of the main one) and hot component (Tapprox.8000 K). All three components have normal abundances. Comparison with ground-based observations or other UV studies reveals the presence on the line of sight of an H II region not detected in the neutral species observed in this study. High-velocity H I gas is also detected, located either in the interstellar medium or very likely in the stellar wind for the blue-shifted components. One of these features is blended with the deuterium lines and therefore obscures our D/H evaluation. All we can say is that the data are compatible with a D/H ratio equal to 1.5 x 10 -5 . This blended feature proved to vary by at least a factor of 3 in column density within few hours, a result which sustains our interpretation of the stellar wind origin of the blueshifted high-velocity H I component. However, the alternative interpretation of a high D/H (approx.10 -4 ) value is also compatible with our data

  20. Mental sundhed i skolen

    DEFF Research Database (Denmark)

    Wistoft, Karen

    Baggrund: Skolebørns opfattelse af sundhed har i høj grad noget med deres selvbilleder, trivsel og sociale inklusion at gøre. Det viser en lang række nyere undersøgelser, der alle peger på, at det er nødvendigt at skærpe opmærksomheden på børn og unges mentale sundhed. Internationalt er der...... udviklet og afprøvet en række pædagogiske programmer, der kan bruges til at igangsætte aktiviteter, der fremmer børn og unges mentale sundhed i skolen. Især i USA, Australien og Storbritannien har der været fokus på evidensbaseret praksis og praksisbaseret evidens i denne sammenhæng. I Danmark har man ikke...... på sammen måde tilgængelig systematiseret viden herom. Sundhedsstyrelsen har gennem de senere år prioriteret indsatser og fremskrivning af evidensgrundlag til fremme af mental sundhed højt. Betydningen af børns mentale sundhed og overvejelser om, hvilken slags evidens der er behov for på dette område...

  1. Housing First i Danmark

    DEFF Research Database (Denmark)

    Benjaminsen, Lars; Dyrby, Therese Marie; Enemark, Morten Holm

    Rapporten er en evaluering af arbejdet med at udbrede og forankre Housing First-indsatsen og tre evidensbaserede bostøttemetoder. Hovedprincippet i Housing First er, at udsatte borgere, der er endt i hjemløshed, får et permanent sted at bo allerede i starten af et indsatsforløb, samtidig med...... at borgeren får en intensiv social støtte i hverdagen. De evidensbaserede bostøttemetoder, der er anvendes sammen med Housing First-tilgangen er Critical Time Intervention (CTI), Intensive Case Management (ICM) og Assertive Community Treatment (ACT). Rapporten beskriver erfaringer omkring de ledelsesmæssige...... og organisatoriske rammer, målgruppen for indsatserne og om de konkrete indsatser i kommunerne. Evalueringen viser, at både borgere og kommuner har gode erfaringer med Housing First, men at især manglen på billige og passende boliger er en alvorlig barriere for udbredelsen af indsatsen. Evalueringen...

  2. Portfolio i Praksis

    DEFF Research Database (Denmark)

    Saltofte, Margit; Krill, Cecilia

    . Præsentationsfolien fokuserer på at præsentere et færdigt produkt eller resultat, mens arbejdsportfolien har fokus på proces, refleksion og læring. Til denne forskel knytter sig desuden helt naturligt en sondring imellem det delte og det private. Hvor præsentationsportfolio per definition er åben og har en modtager i...... sigte, er arbejdsportfolien i udgangspunktet lukket og privat. Det vil sige at man selv udvælger, hvad der skal ses af fx vejleder eller medstuderende – og hvad, der skal forblive privat. Portfolio i Praksis tilbyder desuden en række håndgribelige øvelser, der kan bruges som hjælp til at understøtte det...

  3. Glutaric aciduria type I

    International Nuclear Information System (INIS)

    Mandel, H.; Berant, M.; Braun, J.; Technion-Israel Inst. of Tech., Haifa; El-Peleg, O.; Christensen, E.

    1991-01-01

    Serial CT findings in an infant with glutaric aciduria type I (GA-I) are reported. The major CT features were dilatation of the insular cisterns, regression of the temporal lobes, with 'bat wings' dilatation of the Sylvian fissures and hypodensity of the lenticular nuclei. CT changes preceded the onset of symptoms by 3 months. An improvement in the temporal lobe atrophy was seen after a period of treatment, coinciding with marked clinical improvement. A peculiar feature was the presence of external hydrocephalus, which diverted the attention from manifestations of the primary disease and thus constituted a diagnostic pitfall. The delineation and recognition of the characteristic radiologic manifestations of GA-I are essential for allowing an adequate radiologist/clinician interaction in diagnosing this inborn error of metabolism. (orig.)

  4. Cooperative Learning i voksenundervisningen

    DEFF Research Database (Denmark)

    Wahlgren, Bjarne

    Nationalt Center for Kompetenceudvikling har evalueret undervisningsmetoden Cooperative Learning i voksenundervisningen og dokumenteret positive effekter på oplevelsen af samarbejde og på lærere og kursisters engagement - men har ikke kunnet påvise systematiske positive effekter af metoden på...... kursisters frafald, fravær og karakterer. Projektet har afprøvet og videreudviklet den pædagogiske metode Cooperative Learning (CL) i en dansk virkelighed og mere specifikt i forhold til VUC'ernes nye kursistgrupper med det overordnede mål at øge gennemførslen markant og målbart ved at anvende og udvikle en...

  5. Pindar Pythian I

    Directory of Open Access Journals (Sweden)

    P. Murgatroyd

    1988-05-01

    Full Text Available This article concerns the ancient Greek lyric poet Pindar (approximately 518 to 438 B.C., specifically his first Pythian Ode, and my primary purpose is to supply a new translation of the poem (together with resume and running commentary for illumination and elaboration, thereby introducing Pindar, the nature of his composition and certain features of his style to the non-specialist. In my translation, which is based on the Greek text of Snell & Maehler (1984, I aim above all at accuracy, purity and clarity, and I try to convey to the reader a real idea of the feel and texture of the poem by means of a version which is faithful to the spirit and not just the letter of the Greek. In the essay I provide a résumé, explain the background to the piece, discuss its implications and focus, and treat its major poetic features, such as diction, metre, sound, imagery and structure.

  6. Kognitiv beteendeterapi i skolan

    OpenAIRE

    Nummelin, Maria; Almetun Englund, Britt

    2008-01-01

    Syftet med studien var att få en ökad förståelse och insyn i vad kognitiv beteendeterapi- KBT är, och ta reda på om den stökiga skolmiljön som ofta förekommer, kan förbättras med hjälp av KBT. Våra forskningsfrågor behandlar: Vad är kognitiv beteendeterapi? Vilka förutsättningar krävs bland personalen för att arbeta med KBT vid en skola? När kan det vara lämpligt att pedagogerna i skolan använder sig av KBT? Hur använder pedagogerna KBT i skolan. Studien har genomförts genom bearbetning av re...

  7. Poisonous Properties of Larkspur (<i>Delphinium> spp.

    Directory of Open Access Journals (Sweden)

    Olsen, John D.

    1990-12-01

    Full Text Available Some members of the tribe <i>Delphineae>Warming are useful as forage for grazing cattle or as a source of medicaments for man. However, under certain circumstances their consumption results in poisoning, apparently because of norditerpenoid alkaloids. At least 150 diterpenoid alkaloids have been identified in <i>Delphinium>. In this report, factors influencing the alkaloid content and the toxicity of the plant are discussed. Relative change in content of eight alkaloids at four stages of growth of <i>Delphinium occidentalei> (Watson Watson is reported . The most dominant alkaloids in nine species of <i>Delphinium> are listed. Toxicity of eight species was compared al the flower stage of growth using a mouse assay with <i>D. barbeyii>; (Huth Huth being about four times more toxic than <i>D. glaucescensi> Rydb. or <i>D. geyerii> Greene and about ten times more toxic than <i>Consolida> CV., <i>D. accidentalei>, or <i>Aconitum columbianumi> var. <i>columbianum> Nutt. (vegetative stage. Stress by aphid infestation or relatively low water availability had little if any effect on toxicity of <i>D. barbeyii> and <i>D. occidentalei>, respectively. From chemical analysis of <i>D. barbeyii> tested previously in cattle. the LD50 for a s ingle intraruminal dose of methyllycaconitine was estimated to be less than 6.3 mg/kg body wt.

    [ca] Alguns membres de la tribu <i>Delphineae> Warming són emprats com a farratge per a bestiar de pastura o com a font de medicaments per a l'home. Tanmateix, sota determinades circumstàncies, el seu consum esdevé intoxicació. aparentment per causa d'alcaloides norditerpènics. Al menys 150 alcaloides diterpènics han estat identificats en <i>Delphinium>. En aquest treball es discuteixen els factors que influeixen en el contingut alcalòidic, i, per tant, en la toxicitat de la planla. Es reporten els canvis relatius en el contingut de vuit alcaloides en Quatre estadis de

  8. Forskning i musikterapi

    DEFF Research Database (Denmark)

    Pedersen, Inge Nygaard

    2012-01-01

    . Musikterapi kan dog primært reducere de negative symptomer (herunder emotionel og social tilbagetrukkethed, samt svigtende evner til kontakt og kommunikation). Idet medicin og anden behandling kun i mindre grad indvirker på de negative symptomer, er det væsentligt, at musikterapi påviseligt kan reducere disse....... Antallet af musikterapisessioner er dog vigtigt for effekten, idet 3-10 sessioner kun giver mindre og kortvarige ændringer, hvorimod 16-51 sessioner giver betydningsfulde og længerevarende ændringer. For at fremme patientens motivation og evne til at være i kontakt og kommunikere med andre mennesker...

  9. Strategi i praksis

    DEFF Research Database (Denmark)

    Holm, Michael Jessen; Jenster, Per; Junghagen, Sven

    Præsentation af seks danske små og mellemstore virksomheder, som alle har været succesfulde inden for deres metier. Gennem en belysning af hver virksomheds strategiske situation og udviklingsinitiativer skabes et billede af hvorledes strategi praktiseres i disse virksomheder......Præsentation af seks danske små og mellemstore virksomheder, som alle har været succesfulde inden for deres metier. Gennem en belysning af hver virksomheds strategiske situation og udviklingsinitiativer skabes et billede af hvorledes strategi praktiseres i disse virksomheder...

  10. Sherlock Holmes i arkiverne

    DEFF Research Database (Denmark)

    Lauridsen, Palle Schantz

    2014-01-01

    Er præsentation af, hvordan arkiver og museer har været af afgørende betydning for, at det overhovedet kunne lade sig gøre for forfatteren at finde materiale til bogen "Sherlock Holmes i Danmark".......Er præsentation af, hvordan arkiver og museer har været af afgørende betydning for, at det overhovedet kunne lade sig gøre for forfatteren at finde materiale til bogen "Sherlock Holmes i Danmark"....

  11. Etikk i Norske banker

    OpenAIRE

    Gausdal, Petter Heiskel

    2011-01-01

    Denne oppgaven tar for seg utfordringer knyttet til bruken av etiske retningslinjer i norske banker. Norske banker står ovenfor en fundamental utfordring knyttet til det å levere gode finansielle resultater på en langsiktig og bærekraftig måte slik at de fremstår som en ansvarlig aktør som bidrar til å fremme økonomisk vekst i samfunnet. Bankene er da avhengige av en tillit fra omgivelsene for at de skal få innskudd og dermed grunnlag for videre drift. For å sikre denne tilliten h...

  12. Type I supernova models

    International Nuclear Information System (INIS)

    Canal, Ramon; Labay, Javier; Isern, Jordi

    1987-01-01

    We briefly describe the characteristics of Type I supernova outbursts and we present the theoretical models so far advanced to explain them. We especially insist on models based on the thermonuclear explosion of a white dwarf in a close binary system, even regarding the recent division of Type I supernovae into the Ia and Ib subtypes. Together with models assuming explosive thermonuclear burning in a fluid interior, we consider in some detail those based on partially solid interiors. We finally discuss models that incorporate nonthermonuclear energy contributions, suggested in order to explain Type Ib outbursts. (Author)

  13. Evalueringskultur i praksis

    DEFF Research Database (Denmark)

    Klausen, Martin Søland; Andersen-Mølgaard, Hanna

    Emne: undervisningsmetoder ; evaluering ; folkeskolen ; undervisning ; læring Præsentation og definition af begreber knyttet til arbejdet med evaluering og læring, samt en gennemgang af forskellige evalueringsmetoder og redskaber. Derudover diskuteres de didaktiske overvejelser man bør gøre sig i...

  14. Sociale medier i ungdomslivet

    DEFF Research Database (Denmark)

    Larsen, Malene Charlotte; Lange, Jon Kristian

    2014-01-01

    Brugen af sociale medier er en integreret del af langt de fleste danske unges hverdagsliv. De 16-24-årige er den del af befolkningen, som er kraftigst repræsenteret på sociale medier som Facebook, Instagram, Snapchat og Twitter. I denne aldersgruppe er stort set alle tilknyttet en eller flere...

  15. JLC-I

    International Nuclear Information System (INIS)

    1992-12-01

    The JLC project is the post-Tristan high energy physics program recommended by the High Energy Committee in 1986. The project consists of the construction of a linear collider and the experiment by using it, at an initial center of mass energy around 500 GeV, and eventually it will reach 1.5 TeV along with the successive upgrading of the machine. According to the recommendation, the systematic research and development program was begun, and many remarkable results have been produced. The recent precision measurement at LEP has greatly enhanced the importance of an e + e - linear collider to explore the energy region just above LEP. The construction of the JLC-I is proposed. The main purpose of the JLC-I is to discover and study the Higgs boson and the top quark which are the two missing constituents of the Standard Model. The physics related to them is described. The technical feasibility of the construction of the JLC-I accelerator is considered. The klystrons that can emit the peak power of about 100 MW and experimentally verify the high acceleration gradient of 100 Mv/m are the target of the research and development. The physics, detectors, the accelerator, the time schedule, the cost estimation and others are described. (K.I.)

  16. Talentudvikling i skolen

    DEFF Research Database (Denmark)

    Nissen, Poul; Baltzer, Kirsten

    Effektundersøgelse fra fire udvalgte skoler/projekter, som havde særlige tilbud til deres fagligt stærke elever. De fire talentprojekter gennemførtes ved holddeling inden for den daglige undervisning på henholdsvis 2., 4.-5. og 6.-8 klassetrin i fagene dansk, matematik og projektarbejde, hvor der...

  17. iPark

    DEFF Research Database (Denmark)

    Yang, Bin; Fantini, Ernesto Nicolas; Jensen, Christian S.

    2013-01-01

    where the geo-spatial aspect is not just a tag on other content, but is the primary content, e.g., a city street map with up-to-date road construction data. Along these lines, the iPark system aims to turn volumes of GPS data obtained from vehicles into information about the locations of parking spaces...

  18. Resten i Vesten

    DEFF Research Database (Denmark)

    Moberg, Bergur Rønne

    på formelle elementer ved ikke at privilegere det radikalt nye efter et vestligt-avantgardistisk mønster. Forskellen bliver tydelig i kraft af den ballast af geografi, kultur, historie og politik som de færøske forfattere tænker med, og som afføder en anden udviklings- og originalitetsopfattelse end...

  19. Kanon i dansk 9

    DEFF Research Database (Denmark)

    Thurah, Thomas; Møller, Lene

    Litterær læsning er elevens arbejdsbog til tekstsamlingen Kanon i Dansk 9. den indeholder en grundig introduktion til alle antologiens forfatterskaber, dels baggrundsstof om forfatterne, teksterne og tiden, dels en række opgaver til de enkelte tekster. Bogen er rigt illustreret med billedkunst, som...

  20. Kanon i dansk 9

    DEFF Research Database (Denmark)

    Gyldendals læsebog i kanonlitteratur for 9. klasse. Med sagaer, folkeviser, Højsangen samt tekster af Miguel de Cervantes, Leonora Christina, Thomas Kingo, Ludvig Holberg, Adam Oehlenschläger, N.F.S. Grundtvig, St.St. Blicher, H.C. Andersen, Søren Kierkegaard, Fjodor Dostojevskij, J.P. Jacobsen...

  1. Interessekonflikter i advokatvirksomhed

    DEFF Research Database (Denmark)

    Andersen, Mads Bryde

    2014-01-01

    Gennem 8 teser viser forfatteren, at debatten om advokaters interessekonflikter har overfokuseret på visse hensyn, der taler for at antage en interessekonflikt, men ignoreret andre nok så vigtige hensyn i modsat retning. Dette har ført til regler, der på nogle punkter er uhensigtsmæssige, herunde...

  2. Magien i organisationer

    DEFF Research Database (Denmark)

    Larsen, Mette Vinther

    Magien i organisationer inviterer dig til at se på dit arbejdsliv med nye og nysgerrige øjne. Bogen giver dig mulighed for at reflektere over velkendte organisatoriske, strategiske og ledelsesmæssige udfordringer ud fra et perspektiv, der åbner op for den magi, organisatorisk liv rummer. Dette pe...

  3. BOOKS I BOEKE

    African Journals Online (AJOL)

    fashionable, but not so obviously clear or correct. I picked up the book interested to see whether it could persuade me that the claim makes sense and is true. That someone has a right to the provision of something implies that there is someone else against whom he has this right; and in the case of health care, it is not clear ...

  4. Kontraktetablering i supervision

    DEFF Research Database (Denmark)

    Mortensen, Karen Vibeke; Jacobsen, Claus Haugaard

    2007-01-01

    Kapitlet behandler kontraktetablering i supervision, et element, der ofte er blevet negligeret eller endog helt forbigået ved indledningen af supervisionsforløb. Sikre aftaler om emner som tid, sted, procedurer for fremlæggelse, fortrolighed, ansvarsfordeling og evaluering skaber imidlertid trygh...

  5. iii r r i

    Indian Academy of Sciences (India)

    RESEARCH I NEWS. The 1998 Physics Nobel. Prize. Electrons Behave as if Split into. Three! ... go over some background, starting with the work ofE Hall in ... now exploited all over the world to maintain ... In the same year, the Physics prize ...

  6. College Algebra I.

    Science.gov (United States)

    Benjamin, Carl; And Others

    Presented are student performance objectives, a student progress chart, and assignment sheets with objective and diagnostic measures for the stated performance objectives in College Algebra I. Topics covered include: sets; vocabulary; linear equations; inequalities; real numbers; operations; factoring; fractions; formulas; ratio, proportion, and…

  7. Frivilligcentrene i Danmark

    DEFF Research Database (Denmark)

    Henriksen, Lars Skov

    Rapporten udgør den fjerde og afsluttende delrapport vedrørende evalueringen af 'Puljen til oprettelse af nye og styrkelse af eksisterende frivilligcentre i Danmark'. Udviklingspuljen er en del af sats­pulje­aftalen 2005, hvor satspuljeordførerne besluttede at afsætte 49 millioner kr. til en styr...

  8. Spildesign i Serious Games

    DEFF Research Database (Denmark)

    Helms, Niels Henrik; Uhre Rahbek, Jess

    2012-01-01

    . Artiklen fremhæver udfordringerne ved at skabe koblinger mellem spillets rationalitet og logik i forhold til sammenhænge, hvor det ønskede resultat er læring. De forskellige rationaliteter skaber behovet for en flerfaglig udviklingsproces, men samtidig understreges det, at en sådan proces skaber behov...

  9. Passivhuse i dansk kontekst

    DEFF Research Database (Denmark)

    Ellehauge, Klaus; Thomsen, Kirsten Engelund; Jensen, Ole Michael

    Rapporten redegør for forskelle og ligheder melllem et passivhus, jævnfør passivhuskonceptet fra Passivhaus Institut i Darmstadt, Tyskland og et dansk lavenergihus jævnfør det danske bygningsreglement, ligesom der redegøres for de beregningsprincipper, der ligger til grund. Rapporten redegør endv...

  10. Videnskabsteori i Samfundsvidenskaberne

    DEFF Research Database (Denmark)

    2013-01-01

    Inden for samfundsvidenskaberne findes der mange forskellige og ofte modstridende antagelser om, hvordan viden opbygges, hvad samfundet er, og hvad der indgår i samfundsvidenskabelig analyse. Denne bog sigter på at give en samlende og loyal fremstilling af de mest fremherskende videnskabsteoretiske...

  11. Workshop 95. Part I

    International Nuclear Information System (INIS)

    1995-12-01

    The proceedings of the seminar contain more than 420 contributions divided into 21 areas of interest. In Part I, 144 contributions are presented from the following fields: mathematics, physics, chemistry, engineering informatics and cybernetics, computer art, and fluid mechanics. For input in INIS, 15 contributions were selected from the physics section and 1 from the chemistry section. (J.B.)

  12. POLIOMYELITIS I AIROBI

    African Journals Online (AJOL)

    The age and sex incidence of each racial group are presented in Tables I and n. .... by the selective nature of our admi ions; the great majority of cases from ho pitals .... induced hypothermia, detailed attention to electrolyte and water balances ...

  13. Programmering i praksis

    DEFF Research Database (Denmark)

    Christensen*, Eva Petropouleas; Erkmann, Malene

    ved at bakke ind i. Data bliver konstant samlet ind om os og behandlet af algoritmer, der sender informationer videre til vores telefon, vores bil eller ud på nettet. Den, der kender koderne, har derfor adgang til flere informationer om os, end vi selv har overblik over. Der er mange grunde til...

  14. Human genome I

    International Nuclear Information System (INIS)

    Anon.

    1989-01-01

    An international conference, Human Genome I, was held Oct. 2-4, 1989 in San Diego, Calif. Selected speakers discussed: Current Status of the Genome Project; Technique Innovations; Interesting regions; Applications; and Organization - Different Views of Current and Future Science and Procedures. Posters, consisting of 119 presentations, were displayed during the sessions. 119 were indexed for inclusion to the Energy Data Base

  15. Panik i rummet

    DEFF Research Database (Denmark)

    Pedersen, Peter Ole

    2017-01-01

    Artikel om overvågning og film med fokus på den særlige Home invasion-genre, der er udbredt både i Hollywood-blockbusters, horrorfilm og moderne klassikere. Det psykologiske, statusmæssige og politiske aspekt hos husejeren diskuteres gennem David Finchers 2002-thriller Panic Room....

  16. I-space

    DEFF Research Database (Denmark)

    Helms, Niels Henrik; Tellerup, Susanne; Bryderup, Karin Jensen

    This paper presents I-Space. The purpose of this project is to improve the wellbeing and life quality of mentally impaired citizens through the development of new technologies, which could enhance learning and motivation. The project is used as reference to a discussion on structures within design...

  17. I-space

    DEFF Research Database (Denmark)

    Helms, Niels Henrik; Tellerup, Susanne; Bryderup, Karin Jensen

    2012-01-01

    This paper presents I-Space. The purpose of this project is to improve the wellbeing and life quality of mentally impaired citizens through the development of new technologies, which could enhance learning and motivation. The project is used asreference to a discussion on structures within design...

  18. iCCM

    African Journals Online (AJOL)

    school level (with 6 days statutory iCCM training)2 to be able to manage uncomplicated ... debate that has received less attention has been a failure to demonstrate, or .... qualitative evidence from a pilot study in Tanzania. J Health Commun.

  19. I Mohammed Bilal

    Indian Academy of Sciences (India)

    Home; Journals; Bulletin of Materials Science. I Mohammed Bilal. Articles written in Bulletin of Materials Science. Volume 23 Issue 5 October 2000 pp 425-429 Polymers. Aliphatic amine cured PDMS–epoxy interpenetrating network system for high performance engineering applications—Development and characterization.

  20. iSky

    DEFF Research Database (Denmark)

    Chen, Lijiang; Cui, Bin; Lu, Hua

    2008-01-01

    An interesting problem in peer-based data management is efficient support for skyline queries within a multiattribute space. A skyline query retrieves from a set of multidimensional data points a subset of interesting points, compared to which no other points are better. Skyline queries play an i...

  1. Kvalitet i dagtilbud

    DEFF Research Database (Denmark)

    Christensen, Kira Saabye; Petersen, Mimi; Tireli, Üzeyir

    Denne bog er en grundbog til den specialiseringen Dagtilbudspædagogik på den nye pædagoguddannelse. Bogen bidrager til at udvikle den studerendes generelle vidensgrundlag, studieaktivitet og professionalisering. I bogen anlægges et etisk og videnskabeligt perspektiv på dagtilbudspædagogik og pæda...

  2. Kvalitet I dagtilbud

    DEFF Research Database (Denmark)

    Christensen, Kira Saabye; Petersen, Mimi; Tireli, Üzeyir

    Denne bog er en grundbog til den specialiseringen Dagtilbudspædagogik på den nye pædagoguddannelse. Bogen bidrager til at udvikle den studerendes generelle vidensgrundlag, studieaktivitet og professionalisering. I bogen anlægges et etisk og videnskabeligt perspektiv på dagtilbudspædagogik og pæda...

  3. Workshop 95. Part I

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1996-12-31

    The proceedings of the seminar contain more than 420 contributions divided into 21 areas of interest. In Part I, 144 contributions are presented from the following fields: mathematics, physics, chemistry, engineering informatics and cybernetics, computer art, and fluid mechanics. For input in INIS, 15 contributions were selected from the physics section and 1 from the chemistry section. (J.B.).

  4. Uranium (IV) carboxylates - I

    Energy Technology Data Exchange (ETDEWEB)

    Satpathy, K C; Patnaik, A K [Sambalpur Univ. (India). Dept. of Chemistry

    1975-11-01

    A few uranium(IV) carboxylates with monochloro and trichloro acetic acid, glycine, malic, citric, adipic, o-toluic, anthranilic and salicylic acids have been prepared by photolytic methods. The I.R. spectra of these compounds are recorded and basing on the spectral data, structure of the compounds have been suggested.

  5. Fagreferentkonferansen 2012 i Kristiansand

    Directory of Open Access Journals (Sweden)

    Therese S. Skagen

    2012-09-01

    Full Text Available Universitetet i Agder arrangerte Fagreferentkonferansen 2012 som hadde et innholdsrikt og interessant program. Ulike aspekter ved bibliotekets tjenester ble belyst, fra undervisning av studenter og styrking av informasjonskompetanse, til tjeneste for forskere og forskningsmiljøer og samlingsutvikling med brukerstyrte innkjøp av e-bøker.

  6. Noninvasiv ventilationsbehandling i akutafdelinger

    DEFF Research Database (Denmark)

    Sørensen, Dorthe

    2017-01-01

    også gives som continuous positive airway pressure (CPAP), dvs. en ikke-cyklisk ventilation med et konstant tryk og luftflow, der svarer til en eksspiratorisk modstand, og derfor også er uden egentlig inspiratorisk støtte (2,3). NIV-behandling ved akut respirationssvigt anvendes i dag præhospitalt (4...

  7. Ekspertvirke i organisatorisk kontekst

    DEFF Research Database (Denmark)

    Møller, Bjarke Grønbæk

    2015-01-01

    Kapitlet diskuterer fænomenet ekspertise relateret til en organisatorisk kontekst. Kapitlet er tænkt som en refleksionskilde, der har til formål at kaste lys over, hvordan ekspertise kan forstås, når fænomenet skal ”udfolde” sig i vores organisatoriske hverdagsliv. Med ovennævnte betragtning bliver...

  8. The Internet And I

    Institute of Scientific and Technical Information of China (English)

    曹宇; 陈梅

    2004-01-01

    Several years ago,l was very poor in my lessons,especially in English.l didn't know how to conquer the problem.l was tired of English words,for I was often made to learn thenew words by heart after the other students went home.For a long time my English re-mained weak.

  9. Mobbing i arbeidslivet

    OpenAIRE

    Einarsen, Ståle; Hoel, Helge; Nielsen, Morten Birkeland

    2005-01-01

    Dette heftet er utarbeidet i forbindelse med Arbeidstilsynets prosjektet Jobbing uten mobbing. www.jobbingutenmobbing.no En drøfting av hva mobbing er, hvor omfattende det er, hvilke utslag mobbing gir seg og hvilke konsekvenser det får.

  10. Master i Industriel Informationsteknologi

    DEFF Research Database (Denmark)

    Knudsen, Morten

    2000-01-01

    Målgruppen for denne åbne Masteruddannelse, som udbydes af Aalborg Universitet i samarbejde med IT-Vest, er personer, som dagligt arbejder med teknisk IT, og som har en videregående uddannelse på bachelorniveau. Uddannelsen, som er 3- årig på ½ tid, startede September 1998, og det samlede optag d...

  11. Master i Industriel Informationsteknologi

    DEFF Research Database (Denmark)

    Knudsen, Morten

    2001-01-01

    Målgruppen for denne åbne Masteruddannelse, som udbydes af Aalborg Universitet i samarbejde med IT-Vest, er personer, som dagligt arbejder med teknisk IT, og som har en videregående uddannelse på bachelorniveau. Uddannelsen, som er 3- årig på ½ tid, startede September 1998, og det samlede optag d...

  12. ALMIP Phase I Report

    DEFF Research Database (Denmark)

    Boone, Aaron; de Rosnay, Patricia; Beljaars, Anton

      This report describes the experimental setup and results of the Phase 1 (i.e. the Regional Scale) of the AMMA (African Monsoon Multidisciplinary Analysis) Land surface Model Intercomparison Project (ALMIP). It is part of the AMMA-EU (European Union) and API  (Action Programmée Interorganisme...

  13. Drengeliv i en skoletid

    DEFF Research Database (Denmark)

    Steno, Anne Mia

    2014-01-01

    Doktorgradsprosjektet til Anne Mia Steno fokuserer på møter, gutter har med tekniske yrkesutdannelser, deres framtidsdrømmer og forskjellige navigeringer og strategier på tvers av utdanningssystemet. Denne artikkelen skisserer noen hovedspørsmål i avhandlingen og inneholder også noen metodiske re...

  14. Supplementary Material for: Tukey <i>g-and-h> Random Fields

    KAUST Repository

    Xu, Ganggang; Genton, Marc G.

    2016-01-01

    We propose a new class of transGaussian random fields named Tukey <i>g-and-h> (TGH) random fields to model non-Gaussian spatial data. The proposed TGH random fields have extremely flexible marginal distributions, possibly skewed and/or heavy-tailed, and, therefore, have a wide range of applications. The special formulation of the TGH random field enables an automatic search for the most suitable transformation for the dataset of interest while estimating model parameters. Asymptotic properties of the maximum likelihood estimator and the probabilistic properties of the TGH random fields are investigated. An efficient estimation procedure, based on maximum approximated likelihood, is proposed and an extreme spatial outlier detection algorithm is formulated. Kriging and probabilistic prediction with TGH random fields are developed along with prediction confidence intervals. The predictive performance of TGH random fields is demonstrated through extensive simulation studies and an application to a dataset of total precipitation in the south east of the United States. Supplementary materials for this article are available online.

  15. Ledelsesmodeller i danske virksomheder

    DEFF Research Database (Denmark)

    Kristensen, Kai; Eskildsen, Jacob Kjær; Juhl, Hans Jørn

    2007-01-01

    I de seneste årtier er der opstået flere og flere ledelsesmodeller (kvalitetsprismodeller, ISO 9000, Balanced Scorecard etc.) som virksomheder kan anvende til at opnå en konkurrencemæssig fordel gennem forbedring af forretningsgangene. Rationalet bag de fleste af modellerne er, at der er en...

  16. II!D I

    African Journals Online (AJOL)

    JHypertms 1995; 1J, 91:;'924. 4. Schwartz Gl, Strong Cc. Renal parenchyma! involvement in essential hypertension. Med. Clin Norlh Am 1987; 71, 843-858. 5. Kincaid-5mith P. Malignant hypertension. JHypertms 1991; ~ 893-899. 6. lsaacson C. Milne Fj, van Niekerk I, Kenyon MR, Mzamane DV. The rena1 histopathology of.

  17. Se verden i realtid

    DEFF Research Database (Denmark)

    Koumaditi, Evangelia; Laursen, Camilla Nørskov; Arjomand Kermani, Nasrin

    2018-01-01

    Iværksættervirksomheden ParticleTech arbejder på at skabe et måleinstrument baseret på billedteknologi, der i realtid kan støtteindustrien til at forbedre deres procesoptimering. Dermed åbner der sig en anderledes fremtid for fødevare-, medicinal- og biotekindustrien: Billedanalyse til procesmoni...

  18. Strengere kapitalkrav i pengeinstitutterne

    DEFF Research Database (Denmark)

    Rangvid, Jesper

    2010-01-01

    Artiklen præsenterer estimater over de økonomiske omkostninger ved den finansielle krise vi lige har været igennem. Omkostningerne er meget høje. Kravene om højere egenkapital i banker som Basel III medfører, vil gøre banker sikrere og derved mindske sandsynligheden for fremtidige finansielle...

  19. Tektoniske visioner i arkitektur

    DEFF Research Database (Denmark)

    Beim, Anne

    Essays om 6 berømte arkitekters initiativer i retning af industrialiseret byggeri - afrundet med reflektioner over de fremtidige perspektiver for tektonik, etik og byggeskik. Tektonik handler om udformning og sammenføjning af formelementer til en helhed - om forholdet mellem ide, formprincipper, ...

  20. Feedback i undervisningen

    DEFF Research Database (Denmark)

    Kirkegaard, Preben Olund

    2015-01-01

    undervisningsdifferentiering, feedback på læreprocesser, formativ og summativ evaluering, observationer og analyse af undervisning samt lærernes teamsamarbejde herom. Praktikken udgør et særligt læringsrum i læreruddannelsen. Samspillet mellem studerende, praktiklærere og undervisere giver den studerende en unik mulighed...

  1. ROBINS-I

    DEFF Research Database (Denmark)

    Sterne, Jonathan A C; Hernan, M. A.; Reeves, B. C.

    2016-01-01

    Non-randomised studies of the effects of interventions are critical to many areas of healthcare evaluation, but their results may be biased. It is therefore important to understand and appraise their strengths and weaknesses. We developed ROBINS-I ("Risk Of Bias In Non-randomised Studies - of Int...

  2. Skriften i Skriften

    DEFF Research Database (Denmark)

    Müller, Mogens

    pagtsteologi (1997) 9. Johannesevangeliet og Det Gamle Testamente (offentliggjort på tysk 2003) 10. Historie og teologi. Om afviklingen af Moseloven i Lukasskrifterne (1993) 11. Jesus og Loven (2002; offentliggjort på tysk 2004) 12. "Son of Man" in the Gospels: Quotation, Concept, Or? (2001; ikke tidlgieer...

  3. Akinnibosun, Faith I

    African Journals Online (AJOL)

    Akinnibosun, Faith I. Vol 12, No 2 (2015) - Articles Synergistic effect of Murraya koenigii and Telfairia occidentalis aqueous leaf extract on some bacteria. Abstract. ISSN: 0189-8442. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners ...

  4. Forskningsprojekt i aktivitetsbaserede trafikmodeller

    DEFF Research Database (Denmark)

    Vuk, Goran; Overgård, Christian Hansen

    2011-01-01

    Det Strategiske Forskningsråd ved Programkomiteen for Transport og Infrastruktur bevilgede tre projekter i november 2010 med samlet budget på 54 mil. kr. Et af dem, ACTUM – Analyses of activitybased chains and sustainable mobility, handler om forskning en ny generation af trafikmodeller, såkaldte...

  5. Evolution i overhalingsbanen

    DEFF Research Database (Denmark)

    Abildgaard, Amanda Bering; Hartmann-Petersen, Rasmus; Lindorff-Larsen, Kresten

    2017-01-01

    Vi står på tærsklen til at kunne kurere og måske endda udrydde genetiske sygdomme ved at redigere i menneskets genom. Er det en glidebane mod "designer-babyer" eller et gennembrud på linje med antibiotika og vaccinationer?...

  6. Er HR ude i tovene?

    DEFF Research Database (Denmark)

    Poulfelt, Flemming

    2015-01-01

    HR: Er der behov for nytænkning i HR-land? Artikler i Harvard Business Review - bakket op af en dansk undersøgelse - konkluderer, at HR stadig mangler gennemslagskraft i virksomhederne. Er HR ude i tovene? ... For i undersøgelsen "Ny Dansk Ledelse" (maj 2015), som er baseret på danske lederes...

  7. Arkitektur i "det offentlige billede"

    DEFF Research Database (Denmark)

    Jakobsen, Annette Svaneklink

    2008-01-01

    Igennem de seneste år er visuelle medier blevet en stadig større del af det samlede udtryk der møder os i byer og i bygninger. I asiatiske og i amerikanske storbyer er urbane skærme og medierede facader en almindelig del af det kommercielle og pulserende liv, mens man i Danmark, og resten af Euro...

  8. Unge i kunst- og kulturprojekter

    DEFF Research Database (Denmark)

    Nielsen, Louise Yung; Sørensen, Niels Ulrik

    for ungeaktiviteter i hele landet. Model forsøget, som nu har kørt i tre år, består af 10 projekter, der favner musik, teater, film, litteratur, arkitektur, projektmageri og design. Projekterne foldes ud i ungdomskulturhuse, kulturinstitutioner, folkeskoler og i offentlige rum, hvor unge færdes og lever. I...

  9. 129I Interlaboratory comparison: phase I and phase II

    International Nuclear Information System (INIS)

    Caffee, M. W.; Roberts, M. L.

    1999-01-01

    An interlaboratory comparison exercise for 129 I was organized and conducted. Nine laboratories participated in the exercise to either a full or limited extent. In Phase I of the comparison, 11 samples were measured. The suite of samples contained both synthetic ''standard type'' materials (i.e., AgI) and environmental materials. The isotopic 129 I/ 127 I ratios of the samples varied from 10 -8 to 10 -14 . In this phase, each laboratory was responsible for its own chemical preparation of the samples. In Phase I, the 129 I AMS measurements for prepared AgI were in good agreement. However, large discrepancies were seen in 129 I AMS measurements of environmental samples. Because of the large discrepancies seen in the Phase I 129 I intercomparison, a subsequent study was conducted. In Phase II of the 129 I intercomparison, three separate laboratories prepared AgI from two environmental samples (IAEA 375 soil and maples leaves). Each laboratory used its own chemical preparation method with each of the methods being distinctly different. The resulting six samples (two sets of three) were then re-distributed to the participating 129 I AMS facilities and 129 I/ 127 I ratios measured. Results and discussion of both the Phase I and Phase II interlaboratory comparison are presented

  10. Strategisk ledelse i det offentlige

    DEFF Research Database (Denmark)

    Klausen, Kurt Klaudi

    Bogen opsamler 10 års forskning og undervisning i strategisk ledelse i det offentlige og udvikler en ny koncepttænkning om at se ind i fremtiden og foregribe den gennem de tre F'er......Bogen opsamler 10 års forskning og undervisning i strategisk ledelse i det offentlige og udvikler en ny koncepttænkning om at se ind i fremtiden og foregribe den gennem de tre F'er...

  11. Fixpunkter i Sisimiutstrong />

    DEFF Research Database (Denmark)

    Medonos, Jakub Christensen

    2007-01-01

    Ungdomsklubben i den grønlandske by Sisimiut er et fixpunkt for de unge. Her mødes de på egne præmisser og udfolder sig kreativt. Men der er andet og mere på spil. Det er nemlig gennem fixpunkter som ungdomsklubben, at de unge skaber en bevidsthed om Sisimiut som et særligt byrum.......Ungdomsklubben i den grønlandske by Sisimiut er et fixpunkt for de unge. Her mødes de på egne præmisser og udfolder sig kreativt. Men der er andet og mere på spil. Det er nemlig gennem fixpunkter som ungdomsklubben, at de unge skaber en bevidsthed om Sisimiut som et særligt byrum....

  12. I CROSSED THE LINE

    Directory of Open Access Journals (Sweden)

    Nina Vestby

    2015-12-01

    Full Text Available This article tries to construct a language for my own artistic work with embroidery. Narrative stiches of a young life that was worldly and well traveled into other cultures and norms. About interracial marriage, sickness and living a life less ordinary. Inclusion and exclusion. The connection between body, soul and mind. Art as experience. I look at how these stories and experiences formed my identity with the optics of the German Bildung tradition. Through artistic research and didactics I look into why it is the quality of the embroidery that does so. And will this be transferable to give the youngsters in my multicultural community a way to express their own transcultural lifes.

  13. Performance i Krise

    DEFF Research Database (Denmark)

    Nørreklit, Hanne

    Scorecard got our management team on the same page, and has played an important role in our success thus far( Dieter Zetsche, President and CEO udtaler)."  Den 1. maj 2009 måtte Chrysler indgive sin konkursbegæring. Det er dog ikke kun praksis, der har advokeret nye målemodeller. Mange af de nye...... ikke har kunnet anticipere eller forhindre krisen, men har de mon ligefrem bidraget til krisen? Det konkluderes, at der i modellerne har været indbygget en logik, der har bidraget til en øget risiko og sårbarhed af virksomhederne. Slutteligt diskuteres i hvilken udstrækning, vi som forskere kan og skal...

  14. Arbejdsmarkedsrelationer i Danmark

    DEFF Research Database (Denmark)

    Jensen, Carsten Strøby

    overenskomst- og aftalesystem. Samtidigt indeholder bogen kapitler der belyser konsekvenserne af EU-samarbejdet for det danske arbejdsmarked, ligesom globaliseringens konsekvenser vurderes. Boger er på 318 sider indeholder følgende kapitler. 1. Arbejdsmarkedsrelationer i Danmark, 2. Teorier om de industrielle...... relationer, 3. Faglige organisationer og de industrielle relationer, 4. Arbejdsgiverorganisering og de industrielle relationer, 5. Staten og de industrielle relationer, 6. Overenskomstsystemets grundtræk og udviklingstendenser, 7. Mod nye ledelses-medarbejderrelationer, 8. Komparative perspektiver på...... arbejdsmarkedsrelationerne i Europa, 9. Europæisering af de industrielle relationer, 10. Globaliseringens udfordringer og den danske IR-model, 11. Konklusion - Fra konfliktbaseret konsensus til konsensusbaseret konflikt.   ...

  15. Charles Darwin i 2009

    DEFF Research Database (Denmark)

    Kjærgaard, Peter C.

    2009-01-01

    Charles Darwin blev født d. 12. februar 1809. Hans mest berømte bog, 'Om arternes oprindelse', udkom d. 24. november 1859. Det gør 2009 til noget ganske særligt for både Darwin og evolutionsteorien. Det er nemlig i år både Darwins 200-års fødselsdag og 150-års jubilæet for en af de mest...

  16. Statistics I essentials

    CERN Document Server

    Milewski, Emil G

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Statistics I covers include frequency distributions, numerical methods of describing data, measures of variability, parameters of distributions, probability theory, and distributions.

  17. Science i dagtilbud

    DEFF Research Database (Denmark)

    Broström, Stig; Frøkjær, Thorleif

    Man kan se bogen som en konkretisering af læreplans-temaet "naturen og naturfænomener", og som indgang til et nyt "7. læreplanstema", som måske er på vej! Bogen angiver metoder til at støtte børnene i at undre sig, opstille hypoteser og pædagoger som sammen med børnene konstruerer mulige "naturvi...

  18. Kvalitet i almen praksis?

    DEFF Research Database (Denmark)

    Lippert, Maria Laura; Dyhr, Lise

    2015-01-01

    Artiklen beskriver en undersøgelse, der afdækkede, hvad der kan ske med læge-patient-samtalen, når der i kvalitetssikringsøjemed popper små skærmbilleder op med spørgsmål på lægens PC, der præsenterer faste standarder for indhold og forløb. Konsultationer blandt læger, som anvendte og udfyldte...

  19. I metaforens lys

    DEFF Research Database (Denmark)

    Hansbøl, Gorm

    -as), idet der ikke er tale om at se børnene som nogle andre (ibid. s. 368). At se noget, som-om det er noget andet, er at se wireren som-om, den er en slange i betydningen et krybdyr, eller hvis børnene ses, som-om de er to abeunger, der morer sig. Denne indsigt rummer et stort didaktisk potentiale...

  20. Workshop 96. Part I

    International Nuclear Information System (INIS)

    1995-12-01

    The proceedings of the seminar contain nearly 500 contributions divided into 22 fields of science and technology. In Part I, 167 contributions are presented in the following areas: mathematics, physics, chemistry, engineering informatics and cybernetics, computers, and fluid mechanics. For input in INIS, 19 contributions were selected from the Physics section, 2 from the Mathematics section, and 4 from the Chemistry section. (P.A.)

  1. Workshop 96. Part I

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1996-12-31

    The proceedings of the seminar contain nearly 500 contributions divided into 22 fields of science and technology. In Part I, 167 contributions are presented in the following areas: mathematics, physics, chemistry, engineering informatics and cybernetics, computers, and fluid mechanics. For input in INIS, 19 contributions were selected from the Physics section, 2 from the Mathematics section, and 4 from the Chemistry section. (P.A.).

  2. Risikonavigation i fremdriftsstormen

    DEFF Research Database (Denmark)

    Sarauw, Laura Louise; Madsen, Simon Ryberg

    2017-01-01

    I artiklen bruger vi myten om følgerne af Odysseus’ besøg hos vindenes konge som billede på den med fremdriftsreformen (2014) følgende forestilling om, at det er muligt at sætte en på forhånd fastlagt kurs, hvor blot én vind (et økonomisk rationale) driver de studerende med ét bestemt outcome til...

  3. Til bunds i metropolen

    DEFF Research Database (Denmark)

    Juul, Kristine; Schmidt, Jeanett; Kastanje, Maj

    2012-01-01

    Dette kapitel søger at belyse hvordan vestafrikanske hjemløse, der er kommet til Danmark som arbejdsmriganter klarer sig i København. Pga. vansligheder med at komme ind på det københavnske arbejdsmarked fratages de initiativet som arbejdstagere og overlever istedet som en art moderne jæger-samler......-samler folk gennem flaskesamling og som klienter på diverse sociale tilbud. Denne deroute er forbundet med hvad Goffman har betegnet som stigma....

  4. God skik i forvaltningen

    DEFF Research Database (Denmark)

    Post, Bettina

    2015-01-01

    En række kommuner har forsøgt at hoppe over, hvor gærdet er lavest i sager om gensidig forsørgerpligt for samlevende kontanthjælpsmodtagere. Men det må man ikke. Vi har som borgere ret til at blive hørt og myndighederne har pligt til at opføre sig ordentligt....

  5. Det ustadige i arkitekturen

    DEFF Research Database (Denmark)

    Ryborg Jørgensen, Thomas

    dermed også en højere grad af selvbesindelse og refleksiv frihed, som måske kan føre os videre og åbne for alternativer. Det er en af min afhandlings væsentlige pointer og anfægtelser, at dette ikke kan ske på baggrund af neutrale, ikke-kvalificerede rum, eller rum determineret af/i en simplificeret...

  6. Kirkededikationers betydning i navnebrugen

    DEFF Research Database (Denmark)

    Kællerød, Lars-Jakob Harding

    2015-01-01

    I artiklen diskuteres, om forholdet mellem navne på mandlige patronhelgener for lokale købstadskirker og den lokale navnebrug er så åbenbar, som det ofte er foreslået. Fokus er rettet imod et mindre geografisk område på Sjælland, og undersøgelsens navnekorpus er excerperet fra folketællingen fra ...

  7. Socijalni kapital i prostor

    OpenAIRE

    Ignjatović, Suzana; tomanović, Smiljka

    2012-01-01

    Rad razmatra prostorne aspekte socijalnog kapitala. U prvom delu rada razmatramo kako se prostor shvata u ključnim teorijama socijalnog kapitala. Uključene su tri grupe teorija o socijalnom kapitalu: ključni autori (Bourdieu, Coleman, Putnam), ekonomske/razvojne teorije i mrežne teorije. Analiza pokazuje da autori u različitoj meri shvataju prostor u zavisnosti od svojih teorijskih pristupa socijalnom kapitalu. Drugi deo rada je produbljena komparativna analiza dinamike socijalnog kapitala u ...

  8. Differential equations I essentials

    CERN Document Server

    REA, Editors of

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Differential Equations I covers first- and second-order equations, series solutions, higher-order linear equations, and the Laplace transform.

  9. Familiepleje i Danmark

    DEFF Research Database (Denmark)

    Bryderup, Inge; Engen, Mie; Kring, Sune

    Familiepleje i Danmark handler om den danske familiepleje – om de familier, som vælger at blive familieplejere, og om de børn og unge, der er anbragt hos dem. Siden slutningen af 1980’erne har familiepleje været den mest anvendte anbringelsesform, når det gælder socialt udsatte børn og unge. Vi v...

  10. Faglighed i forandring

    DEFF Research Database (Denmark)

    Nielsen, Carsten Tage

    2009-01-01

    En kritisk læsning af Faghæftet: Fælles Mål 2009: Historie. Faghæftens anvisninger i de bidende tekster og de vejledende tekster er modsigelsesfyldte. To fagsyn gør sig gældende og strides mod hinanden. Et taler for absolut kronologi, et andet for relativ kronologi. Faghæftet har ikke ført til den...

  11. Vikingeborgen i Limfjordslandet

    DEFF Research Database (Denmark)

    Sindbæk, Søren Michael

    2013-01-01

    . Arkæologien har vist, at borgene blev bygget omkring eller kort før år 980, mens Harald Blåtand var konge, og så godt som sikkert på hans befaling. Hverken før eller siden fik folk i vikingetidens Danmark noget lignende at se. Hvordan blev et byggeri som dette muligt? For nylig er det lykkedes at kaste nyt...

  12. Extragalatic zoo. I

    International Nuclear Information System (INIS)

    Schorn, R.A.

    1988-01-01

    The characteristics of various types of extragalactic objects are described. Consideration is given to cD galaxies, D galaxies, N galaxies, Markarian galaxies, liners, starburst galaxies, and megamasers. Emphasis is also placed on the isolated extragalatic H I region; the isolated extragalatic H II region; primeval galaxies or photogalaxies; peculiar galaxies; Arp galaxies; interacting galaxies; ring galaxies; and polar-ring galaxies. Diagrams of these objects are provided

  13. Turisme i landdistrikter

    DEFF Research Database (Denmark)

    Hjalager, Anne-Mette

    1998-01-01

    en erhvervsmæssig aktivitet og deraf følgende jobs og indkomster. Samtidig med de optimistiske forventninger udtrykkes der også forbehold. Kan turismen leve op til den rolle som den tillægges? Artiklen viser, at landdistrikterne også opnår vækst som følge af turisme, men at øerne i de senere år har...... ikke er relevant at tale om stabile jobs....

  14. [125I]Iodopride

    International Nuclear Information System (INIS)

    Janowsky, A.; Ebert, M.H.; De Paulis, T.; Vanderbilt Univ., Nashville, TN; Kessler, R.M.; Vanderbilt Univ., Nashville, TN; Clanton, J.A.; Smith, H.E.

    1988-01-01

    Substituted benzamides are currently among the most selective antagonists at dopamine D-2 receptors, and high affinity ligands have been developed by substituting halogens into the aromatic ring of the benzamides. The authors report the high affinity, stereoselective, reversible, and sodium dependent binding of a new iodine-substituted benzamide, called [ 125 I]iodopride, to a membrane preparation from rat corpus striatum. 5 refs.; 1 figure

  15. Business statistics I essentials

    CERN Document Server

    Clark, Louise

    2014-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Business Statistics I includes descriptive statistics, introduction to probability, probability distributions, sampling and sampling distributions, interval estimation, and hypothesis t

  16. Godt i gang

    DEFF Research Database (Denmark)

    Bruun Andersen, Kirsten; Bisgaard, Hanne; Carstensen, Susanne

    Bogen er et materiale til undervisning af let øvede brugere - døvblevne, svært hørehæmmede og hørende - i tegnstøttet kommunikation. Materialet er en fortsættelse af ´Godt begyndt´, og gloseforrådet fra ´Godt begyndt´ forudsættes kendt...

  17. Computer science I essentials

    CERN Document Server

    Raus, Randall

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Computer Science I includes fundamental computer concepts, number representations, Boolean algebra, switching circuits, and computer architecture.

  18. Liv i universet

    DEFF Research Database (Denmark)

    Rasmussen, Ib Lundgaard

    Liv i universet - er vi alene? Dette store spørgsmål forsøger den nye videnskab astrobiologi at svare på. Udviklingen af liv her på Jorden danner nemlig grundlag for vores søgen efter liv andre steder. Gennem studiet af livets opståen og udvikling på Jorden kan man opstille retningslinjer for...

  19. Integration i multikulturelle folkeskoler

    DEFF Research Database (Denmark)

    Gitz-Johansen, Thomas

    folkeskolen nye befolkningsgrupper, som ikke har afskaffet ”gamle” sociale og regionale skel, men som i mange skoler har ført til en situation, der bedst kan beskrives som multikulturel. Denne beskrivelse skal ikke opfattes som en pædagogisk programerklæring men som en beskrivelse af elev-grundlaget og i....... Dette design har haft sine omkostninger hvad angår dybden i analyserne af de enkelte klasser og elev-grupper, men til gengæld har det givet et bredere udsyn til to skoler og til en række elever, lærere og pædagoger og deres indbyrdes interaktioner. Det ”mini-longitudinelle” design har desuden givet mig...... tilgang er, at kulturelle identiteter ikke kan forstås som medfødte eller naturlige egenskaber ved en given befolkningsgruppe, men må opfattes som identiteter, der er blevet til og reproduceres gennem et fortløbende konstruktionsarbejde, der blandt andet indebærer at beskrive og lægge afstand til andre...

  20. NITRATI I NITRITI, METABOLIZAM I TOKSIČNOST

    OpenAIRE

    Nujić, Marija; Habuda-Stanić, Mirna

    2017-01-01

    Nitrati i nitriti su kemijski spojevi koji se koriste kao gnojivo, rodenticidi ili konzervansi. Mogu se naći u zraku, tlu, vodi ili hrani (posebice povrću) a mogu se i stvarati u ljudskom tijelu. Nitrati imaju važnu ulogu u hranjenju i metabolizmu biljaka. Nastaju oksidacijom organskog otpada djelovanjem dušičnih bakterija. Ljudi mogu biti izloženi nitratima i nitritima preko konzumacije povrća i mesnih prerađevina, a manjim dijelom preko vode ili ostale hrane. Pijenje vode sa već...

  1. MRI findings in the mild type of mucopolysaccharidosis II (Hunter's syndrome)

    International Nuclear Information System (INIS)

    Shimoda-Matsubayashi, S.; Ito, T.; Hattori, N.; Okuma, Y.; Mizuno, Y.; Kuru, Y.; Sumie, H.

    1990-01-01

    Neuroradiological findings in a 44-year-old male with the typical mild type of Hunter's disease are reported. Cranial MRI revealed patchy areas of increased and decreased signals in T1- and T2-weighted images in the thalamus and the basal ganglia giving rise to a honey comb-like appearance as a whole. The deep white matter showed high signals in the T2-weighted image. To our knowledge, the honey comb-like appearance has never been reported in this disorder. Deposition of mucopolysaccharides and/or glycolipids and increase in fluid content seem to be responsible for these changes. (orig.)

  2. The thesis of judicialization of health care by the elites: medication for mucopolysaccharidosis

    OpenAIRE

    Medeiros, Marcelo; Diniz, Debora; Schwartz, Ida Vanessa Doederlein

    2013-01-01

    O artigo avalia a hipótese de se a judicialização de medicamentos para o tratamento das mucopolissacaridoses no Brasil seria uma ação das elites econômicas. Debatem-se estudos prévios que defendem a tese da judicialização pelas elites em outros medicamentos. Discute-se, a metodologia desses estudos e as inferências dela derivadas e o respaldo empírico dessa tese no caso de um dos medicamentos judicializados de mais alto custo para o SUS. Foram analisados os 196 proc...

  3. Wittgenstein i zagadka Anzelma

    Directory of Open Access Journals (Sweden)

    Jakub Gomułka

    2013-09-01

    Full Text Available The paper is devoted to the problem of the two paradoxes: the paradox of absolute value in Wittgenstein’s Lecture on Ethics and the paradox of Anselm’s Name of God from the Proslogion. I try to present linguistic determinants of both paradoxes and point out to certain similarities between them. However, the main part of the article focuses on the solution of Anselm’s paradox given by Cora Diamond, the prominent Wittgensteinian scholar in her 1995 book The Realistic Spirit. Diamond develops extensive comparison of the concept of that than which nothing greater can be conceived and a series of peculiar riddles – she calls the Anselm’s concept the great riddle, since, as in other riddles presented, we cannot deal with it in a usual way. The author of The Realistic Spirit shows that what enables us to resolve, say, the riddle of Sphinx, is our obtaining a new way of understanding of the words which compose the riddle itself, because the standard understanding will not lead us to any answer. However, when it comes to the great riddle we know that we cannot obtain any way of understanding which will enable us to conceive it. This does not mean that the riddle cannot make any sense for us. We can believe that there is a solution (in fact, we know the solution which has been given to us in the Revelation, but we cannot understand it and hence we can treat the great riddle as an euqation which we lack proof but we know there is one. As Diamond calls it, we have the promissory meaning of the riddle. In my opinion the idea of the promissory meaning as an interesting solution of Anselm’s paradox, but I notice that the idea itself is conditioned by religious faith or by something I call the empathic atheism (in short: an attitude which let atheists to imagine the believers’ point of view.

  4. Metoder i livskvalitetsforskning

    DEFF Research Database (Denmark)

    Grønvold, Mogens

    2008-01-01

    Der er udviklet en omfattende forskningsmetodologi til belysning af den videnskabelige kvalitet af spørgeskemaer om helbredsrelateret livskvalitet. Da denne metodologi kan udforske vigtige aspekter af spørgeskemaers validitet og reliabilitet, er det centrale spørgsmål ikke længere, om spørgeskema...... spørgeskemaundersøgelser er valide, men om, hvor tilstrækkeligt deres validitet ved givne anvendelser er undersøgt. Med nyere statistiske metoder ses et skift i retning af interaktive, individualiserede spørgeskemaer. Udgivelsesdato: 2008-Mar-3...

  5. Filaments in Lupus I

    Science.gov (United States)

    Takahashi, Satoko; Rodon, J.; De Gregorio-Monsalvo, I.; Plunkett, A.

    2017-06-01

    The mechanisms behind the formation of sub-stellar mass sources are key to determine the populations at the low-mass end of the stellar distribution. Here, we present mapping observations toward the Lupus I cloud in C18O(2-1) and 13CO(2-1) obtained with APEX. We have identified a few velocity-coherent filaments. Each contains several substellar mass sources that are also identified in the 1.1mm continuum data (see also SOLA catalogue presentation). We will discuss the velocity structure, fragmentation properties of the identified filaments, and the nature of the detected sources.

  6. Physics I essentials

    CERN Document Server

    REA, The Editors of

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Physics I includes vectors and scalars, one-dimensional motion, plane motion, dynamics of a particle, work and energy, conservation of energy, dynamics of systems of particles, rotation

  7. Thermodynamics I essentials

    CERN Document Server

    REA, The Editors of

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Thermodynamics I includes review of properties and states of a pure substance, work and heat, energy and the first law of thermodynamics, entropy and the second law of thermodynamics

  8. Java I/O

    CERN Document Server

    Harold, Elliotte Rusty

    2006-01-01

    All of Java's Input/Output (I/O) facilities are based on streams, which provide simple ways to read and write data of different types. Java provides many different kinds of streams, each with its own application. The universe of streams is divided into four largecategories: input streams and output streams, for reading and writing binary data; and readers and writers, for reading and writing textual (character) data. You're almost certainly familiar with the basic kinds of streams--but did you know that there's a CipherInputStream for reading encrypted data? And a ZipOutputStream for automati

  9. Electronics I essentials

    CERN Document Server

    REA, The Editors of

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Electronics I covers fundamentals of semiconductor devices, junction diodes, bipolar junction transistors, power supplies, multitransistor circuits, small signals, low-frequency anal

  10. Group theory I essentials

    CERN Document Server

    Milewski, Emil G

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Group Theory I includes sets and mapping, groupoids and semi-groups, groups, isomorphisms and homomorphisms, cyclic groups, the Sylow theorems, and finite p-groups.

  11. Det ligger i luften

    DEFF Research Database (Denmark)

    Klitmøller, Anders; Lauring, Jakob; Jonsen, Karsten

    2011-01-01

    Multikulturelle virtuelle teams er en udbredt samarbejdsform i større danske virksomheder, specielt til løsning af videns intensive og kreative opgaver. Som leder kan man umiddelbart opnå fleksibilitet og besparelser ved brug af virtuelle teams, men desværre bliver disse fordele ofte spoleret af ......’ medier, såsom telefon og videokonference, ikke altid er effektive til kreative opgaver, da sproglig og kulturel friktion kan forøges ved brug af disse. De forskningsmæssige implikationer af studiet diskuteres, og ledelsestiltag, som imødegår disse udefordringer udfordringer, belyses....

  12. Processuel strategi i organisationer

    DEFF Research Database (Denmark)

    Gylling, Martin

    2014-01-01

    on process theory and strategy, which in these years aims to move focus away from the dominance of designed strategy. I.e. an approach to practice understood as methodological individualism and pragmatic rationality (Chia & Holt 2009; Steyaert 2007; Stacey 2011; Hjorth 2012). Through recent poststructuralist...... currents in the field of processual strategy thinking and organisational entrepreneurship, for example Chia & Holt’s creation philosophical research in strategy without design and Steyaert’s (2007) thinking on the concept of entrepreneuring; the process theoretical thinking of entrepreneurship...

  13. Algebra & trigonometry I essentials

    CERN Document Server

    REA, Editors of

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Algebra & Trigonometry I includes sets and set operations, number systems and fundamental algebraic laws and operations, exponents and radicals, polynomials and rational expressions, eq

  14. Who am I?

    DEFF Research Database (Denmark)

    Smedegaard Ernst Bengtsen, Søren

    What does it mean to grasp or frame the singular level of the self? The difficulties in exploring and laying bare the singular dimension of the self have haunted qualitative research for several years. The literature on supervision in higher education has for the last three decades been occupied...... with research into the personal dimension of the supervisory dialogue (Bengtsen, 2011; Wisker, 2005). However, this research defaults to descriptions of the personal dimension through the use of general types, categories and conversational patterns. In my own research I explore the relation between general...

  15. Geometry I essentials

    CERN Document Server

    REA, The Editors of

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Geometry I includes methods of proof, points, lines, planes, angles, congruent angles and line segments, triangles, parallelism, quadrilaterals, geometric inequalities, and geometric

  16. Transport phenomena I essentials

    CERN Document Server

    REA, The Editors of

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Transport Phenomena I includes viscosity, flow of Newtonian fluids, velocity distribution in laminar flow, velocity distributions with more than one independent variable, thermal con

  17. Data structures I essentials

    CERN Document Server

    Smolarski, Dennis C

    2012-01-01

    REA's Essentials provide quick and easy access to critical information in a variety of different fields, ranging from the most basic to the most advanced. As its name implies, these concise, comprehensive study guides summarize the essentials of the field covered. Essentials are helpful when preparing for exams, doing homework and will remain a lasting reference source for students, teachers, and professionals. Data Structures I includes scalar variables, arrays and records, elementary sorting, searching, linked lists, queues, and appendices of binary notation and subprogram parameter passi

  18. Performance management i en krisetid

    DEFF Research Database (Denmark)

    Elmholdt, Claus Westergård; Hoffmann Birk, Rasmus; Børgesen, Kenneth

    2015-01-01

    Denne artikel undersøger, om ’performance management’ – eller på dansk præstationsledelse – er et effektivt svar på de udfordringer, som organisationer står overfor i krisetider. I tiden efter finanskrisen i september 2008 er det blevet endnu mere relevant for ledere at kunne navigere i en...

  19. Uanmodet kommunikation i sociale medier

    DEFF Research Database (Denmark)

    Trzaskowski, Jan

    2012-01-01

    henvendelser ikke omfatter beskeder i sociale medier. Indtil den 12. juni 2013 kan man i national ret opretholde en bredere definition af begrebet elektronisk post i lyset af fjernsalgsdirektivet. Herefter skal spørgsmålet om uanmodet elektronisk post i sociale medier vurderes efter handelspraksisdirektivet....

  20. Tillid i den finansielle sektor

    DEFF Research Database (Denmark)

    Rimmer, Nina Røhr; Nielsen, Sara Møller; Benediktsdottir, Elisabet

    2014-01-01

    , der er i kommunikation med kunden, eksempelvis at videreudvikle på definerede Touch-Points i CEM og opbyggelsen/vedligeholdelsen af kundeoplysninger i CRM systemer således, at den samlede kvalitet af ydelsen bliver så personlig som mulig, hvilket falder godt i tråd med Spar Nords værdigrundlag om dels...

  1. I Frans af Assisis fodspor

    DEFF Research Database (Denmark)

    Bonde, Lisbeth

    2015-01-01

    En fodrejse fra Assisi til Rom i Frans af Assisis fodspor sætter gang i tankerne og giver anledning til nye ideer......En fodrejse fra Assisi til Rom i Frans af Assisis fodspor sætter gang i tankerne og giver anledning til nye ideer...

  2. HRM i Danmark efter krisen

    DEFF Research Database (Denmark)

    Bévort, Frans; Holt Larsen, Henrik; Hjalager, Anne-Mette

    Kortlægning af praksis for Human Resource Management (HRM) i alle organisationer i Danmark med mere end 100 ansatte.......Kortlægning af praksis for Human Resource Management (HRM) i alle organisationer i Danmark med mere end 100 ansatte....

  3. Detailudformning af cykelstier i kryds

    DEFF Research Database (Denmark)

    Fjordback, Michael; Lahrmann, Harry; Sørensen, Michael

    2007-01-01

    Dette paper er baseret på specialet "Cykelstiers udformning i kryds - Analyse baseret på skadestueregistrerede uheld" (Fjorback 2007), der er udarbejdet i perioden 1. februar - 12. juli 2007 ved Aalborg Universitet på civilingeniøruddannelsen i vej- og trafikteknik. I specialet er der fokuseret p...

  4. iPad portable genius

    CERN Document Server

    McFedries, Paul

    2013-01-01

    Make the most of your iPad with these savvy tips and techniques The iPad, iPad Air, and iPad mini are taking the world by storm. This no-nonsense guide covers everything you want to know to take full advantage of all your iPad has to offer. Along with necessary basics about the newest iPad; iPad Air, iPad mini; and iOS; it gives you smart; innovative ways to accomplish a variety of tasks and tips to help you maximize the convenience of your Apple digital lifestyle. More than 100 million iPads have been sold; and the number continues to growHip and practical; Portable Genius guides are packed

  5. Integration i flere variable

    DEFF Research Database (Denmark)

    Markvorsen, Steen

    2010-01-01

    Denne note handler om parameterfremstillinger for kurver, flader og rumlige områder og om integration af funktioner på sådanne geometriske objekter. Formålet er primært at opstille og motivere de generelle definitioner og beregninger af henholdsvis kurve- \\, flade- \\, og rum-integraler. Udgangspu......Denne note handler om parameterfremstillinger for kurver, flader og rumlige områder og om integration af funktioner på sådanne geometriske objekter. Formålet er primært at opstille og motivere de generelle definitioner og beregninger af henholdsvis kurve- \\, flade- \\, og rum......-integralerne. Undervejs introduceres \\texttt{Integrator8}. Det er en pakke med Maple procedurer, som er udviklet specielt med henblik på eksempelbaseret visuel læring af de indledende integrationsbegreber og deres mangfoldige anvendelser. Vi giver eksempler på, hvordan integration i flere variable anvendes til beregning...... og forståelse af rumfang, vægt, massemidtpunkter, inertimomenter, kraftmomenter, etc. Flowkurverne for et givet vektorfelt i rummet kan findes og visualiseres med \\texttt{Integrator8}. De vigtige begreber divergens og rotation for et vektorfelt fremtræder derved som naturlige størrelser til...

  6. Father and I

    Science.gov (United States)

    Yang, Chen Ning

    2013-05-01

    Father (K. C. Yang (楊克純), 1896-1973) was a high school teacher in Anqing (安慶) in 1922 when I was born in Hefei (合肥). Anqing was then also called Huaining (懷寧). Father gave me the name Chen Ning, of which Chen was the name of my generation in our family, and Ning was derived from Huaining. Before I was one year old Father won an Anhui (安徽) Provincial Fellowship for studying in the USA. We had a family picture (Figure 1) taken in the courtyard outside our bedroom a few days before he left home. Father had on the traditional robe and coat, standing stiff and erect. He had probably up to that point never worn a western suit. Two years later he sent a picture (Figure 2) to Mother from the University of Chicago, in which his attire and bearing had both entered the twentieth century. Father was a handsome man. The exuberance and optimism of his youth were clearly captured in this photograph...

  7. Statyny i astma

    Directory of Open Access Journals (Sweden)

    Joanna Pawlak

    2011-01-01

    Full Text Available Statyny należą do leków powszechnie stosowanych w leczeniu hiperlipidemii oraz chorób układu sercowo-naczyniowego. Hamując aktywność reduktazy 3-hydroksy-metyloglutarylo-koenzymu A (HMG-CoA zmniejszają syntezę cholesterolu. Wykazano, że leki te cechują się znacznie szerszym zakresem działania, które określono jako pozalipidowe (plejotropowe. Mimo że korzyści z działania inhibitorów reduktazy HMG-CoA zostały już dowiedzione w leczeniu chorób układu sercowo-naczyniowego, podejmuje się próby ich wykorzystywania w innych dziedzinach medycyny, takich jak neurologia czy reumatologia. Obecnie szczególną uwagę zwraca się na działanie przeciwzapalne i immunomodulujące tych leków. Na podstawie powyższych obserwacji w ostatnich latach podjęto próby zastosowania statyn również w alergologii.W pracy przedstawiono wybrane aspekty wpływu statyn na reakcje immunologiczne i proces zapalny wskazując na możliwość wykorzystania tych leków w leczeniu astmy.

  8. Pædagogen i skolen

    DEFF Research Database (Denmark)

    Dupont, Stine; Hansen, Maj Borggaard; Kofoed-Heller, Klaus

    2017-01-01

    Pædagoger i skolen oplever, at de i skolen skal agere professionelt på en anden måde end de er vant til. De oplever, at de står spændt ud mellem forskellige logikker i henholdsvis skole- og fritidsinstitutioner. Dette prøver vi at indfange ved at vise, at der er forskellige logikker i spil i...

  9. Dødsbrande i Danmark

    DEFF Research Database (Denmark)

    Leth, Peter Mygind

    2006-01-01

    rapporten fremgår det om dødsbrande, at: • Det årlige antal af omkomne i brand har ligget på gennemsnitligt 83 personer om året i perioden 2000 - 2005. • De fleste af de omkomne i brand er mænd, således omkom dobbelt så mange mænd i brand som kvinder i 2005. • Alder er en væsentlig faktor. Jo ældre man er......Denne rapport indeholder en gennemgang og analyse af dødsbrande i Danmark i perioden 2000-2005. Analysen bygger primært på talmateriale, der stammer fra Beredskabsstyrelsens indsamling af oplysninger om dødsbrande. I rapporten beskrives desuden, hvordan antallet af omkomne i brand kan nedbringes. I......, jo højere risiko har man for at omkomme i brand. • Tobaksrygning udgør den største enkeltårsag til dødsbrande. • Hovedparten af de branddøde omkommer i forbindelse med brande i boligen. Antallet af plejehjemsbeboere og beboere i beskyttede boliger er overrepræsenterede i dødsbrandstatistikken. • De...

  10. Wavelengths and energy levels of I V and I VI

    International Nuclear Information System (INIS)

    Kaufman, V.; Sugar, J.; Joshi, Y.N.

    1988-01-01

    The spectra of iodine were photographed in the 139--1500-Aat;O region on various spectrographs. Earlier analyses of I V and I VI were revised and extended. For I V 26 lines were classified, and for I VI 35 lines were classified. Ionization energies have been estimated to be 415 510 atm≅ 300 cm -1 (51.52 atm≅ 0.04 eV) and 599 800 atm≅ 3 000 cm -1 (74.37 atm≅ 0.37 eV) for I V and I VI, respectively

  11. Experiments around I-8

    CERN Multimedia

    CERN PhotoLab

    1975-01-01

    The lithium transition-radiation detectors and the large liquid argon calorimeters of experiment R806T are shown above and below the intersection at I-8 (Brookhaven-CERN-Saclay-Syracuse-Yale Collaboration, Study of large transverse momentum phenomena by electron and photon detection). At 90 deg to the intersecting beams are the monitoring proporional chambers of experiment R805 (Measurement of real to imaginary ratio of forward scattering amplitude - Coulomb interference - by the CERN-Rome Collaboration). Left and right of the intersection one sees, symmetrically placed around the interaction region, the large scintillation counters hodoscopes used by R801 (Pisa-Stony Brook Collaboration) to measure the pp total cross section and the features of inelastic collisions.

  12. Dismantling of Vandellos I

    International Nuclear Information System (INIS)

    Armada, J. R.

    2003-01-01

    Spain is witnessing the phase-out of a nuclear power plant. It is a unique experience in our country and therefore the dismantling work has been watched closely, not only from here but also from abroad. The Empresa Nacional de Residuos Radiactivos (ENRESA) is in charge of managing the dismantling and decommissioning work of the Vandellos-I nuclear power plant, located in the municipality of L'Hospitalet de l'Infant (Tarragona). the work began five years ago and has been executed on schedule. the appearance of what was one of the first Spanish commercial nuclear power plants has been changed radically to leave premises suitable for any other activity. (Author)

  13. Prostitution i Danmark

    DEFF Research Database (Denmark)

    Kofod, Jens Erik; Frøkjær Dyrvig, Theresa; Markwardt, Kristoffer

    Denne rapport præsenterer resultaterne af SFI’s kortlægning af prostitution i Danmark. Rapporten belyser omfanget af prostitution og de prostitueredes levevilkår. Rapporten er dels baseret på en omfattende kvalitativ interviewundersøgelse blandt nuværende og tidligere prostituerede samt relevante...... livssituation særligt er knyttet til, om de føler sig økonomisk afhængige eller uafhængige af penge fra prostitution, og om de føler sig socialt inkluderet eller ekskluderet af samfundet. Kortlægningen viser, at gruppen af prostituerede er meget heterogen, og at de prostituerede har meget forskellige ønsker og...

  14. Alex & I: Against Indifference

    Directory of Open Access Journals (Sweden)

    Sumugan Sivanesan

    2016-03-01

    Full Text Available This text and photo essay concerns a series of portraits made with a community of Tamil refugees living in Bangkok who refer to themselves as ‘the Bachelors.’ The project was initiated by refugee and one-time media figure, Sanjeev ‘Alex’ Kuhendrarajah who hoped his peers would tell their own stories to an ‘international community.’ With reference to Judith Butler’s Frames of War (2009, I have sought to ‘discursively frame’ the images by considering the discrimination these young single men encounter living in the margins of this South Asian metropolis, awaiting the outcomes of their re-settlement applications.

  15. I-Introduction

    International Nuclear Information System (INIS)

    1998-01-01

    In this report, each sector starts with an introduction presenting the state of each sector in Lebanon, followed by the methodology adopted in order to compute emissions of greenhouse gases by sources and removals by sinks, in accordance with IPCC guidelines, complemented by experts' assumptions in estimating greenhouse gases and finally accompanied by the IPCC sectoral tables which present the result obtained in each sector. The report ends with an 'Appendices' chapter which includes all worksheets, showing the computations made resulting in emitted or removed greenhouse gas quantities. This executive summary described in the IPCC summary presents the main results obtained in each sector. In the last paragraph, the global warming potential of the greenhouse gases emitted in Lebanon in 1994 is provided for three calculated time horizons, based on the '1995 IPCC technical summary of working group I' report. (au)

  16. Talent i skolen

    DEFF Research Database (Denmark)

    Nissen, Poul Erik

    Talent i skolen inddrager den nyeste danske og internationale forskning inden for talentpleje og byder ind med initiativer, der kan støtte op om de særligt begavede børn. Gennem konkrete cases og introduktion af begrebet "potentialeorienteret undervisningsdifferentiering" får læreren inspiration...... til planlægning og undervisning af fagligt indhold, der også udfordrer de særligt begavede elever. Endelig præsenteres en praksisnær ramme for identifikation og beskrivelse af børn, der antages at have særlige talenter eller forudsætninger samt forslag til, hvorledes man kan sikre, at talentudvikling...

  17. iMac portable genius

    CERN Document Server

    Hart-Davis, Guy

    2010-01-01

    The most up-to-date coverage on the latest iMac advice, tools, and shortcuts Cool and useful tips, full-color screenshots, and savvy advice show you how to get the most out of your iMac. Fully updated to cover the iMac's latest features and capabilities, this guide is packed with indispensible information on iLife '09 and Mac OS X Snow Leopard, and shows you how to customize your iMac in a way that it will work best for you.Explores all the bells and whistles of the iMac, including the new Magic Mouse, iLife apps such as iPhoto and iMovie, and Mac OS X Snow LeopardShows yo

  18. Mindfulness i skolen : ABC, 123, samt elever i balanse

    OpenAIRE

    Kristiansen, Kristin

    2011-01-01

    I oppgaven redegjøres for begrepet og tilstanden mindfulness. Både mindfulness med opphav i Østens kontemplative tradisjon, og Ellen Langers vestlige mer kognitive mindfulness, defineres og utdypes. Videre diskuteres mindfulness i en pedagogisk sammenheng. Siste del av oppgaven er en spørreundersøkelse, der lærerne i Tromsøskoler sine erfaringer, holdninger og interesser relatert til kontemplativ pedagogikk og mindfulness, er kartlagt.

  19. iMac pocket genius

    CERN Document Server

    Hart-Davis, Guy

    2010-01-01

    If you want to get the very most out of your iMac, put this savvy Portable Genius guide to work. Want to make the most of the new Magic Mouse and the latest iLife apps? Set up a wireless network using your iMac's AirPort card? Watch television on your iMac, or show iMac videos and movies on your television? You'll find cool and useful Genius tips, full-color screenshots, and pages of easy-to-access shortcuts and tools that will save you time and let you enjoy your iMac to the max.

  20. Viis aastat iPhone’i / Tõnu Ojala

    Index Scriptorium Estoniae

    Ojala, Tõnu, 1969-

    2012-01-01

    Jaanuar ajaloos: 9. jaanuaril 2007. aastal esitles Apple'i juht Steve Jobs esimest iPhone'i. 13. jaan. 1942 päästis Saksa katselendur Helmut Schenck esimese lendurina oma elu katapulteerumisega. 12. jaan. 1907 sündis Sergei Koroljov

  1. Sygehusene i den islamiske verden i den klassiske tid

    OpenAIRE

    Provencal, Philippe

    2014-01-01

    Et af de væsentlige punkter, hvor den arabiske medicin udmærker sig i forhold til tidligere tiders medicin var i oprettelsen og organiseringen af regelrette sygehuse. Artiklen beskriver sygehusenes oprindelse, plads og funktion i den klassiske arabiske kultur (750-1500)

  2. Beyond iPS!

    Directory of Open Access Journals (Sweden)

    Editorial

    2012-01-01

    Full Text Available It’s undoubtedly a jubilant moment for scientists and clinicians working in the stem cell arena as Prof. Gurdon and Prof. Shinya Yamanaka have been chosen for the Nobel Prize in Physiology & Medicine this year. The mystery of cell biology is something unfathomable and probably the work of this duo as well as the other scientists, who have put their hands on in- vitro de-differentiation have opened our eyes to a new window or a new paradigm in cell biology. The iPS invention has brought a lot of hope in terms of potential direct benefits to treat several diseases, which have no definite options at the moment. But, we envisage that several spin-offs could come out of this invention and one very significant spin-off finding recently witnessed is the finding by Prof. Masaharu Seno and his team of researchers at the Okayama University, Japan (Chen L, et al. 2012, PLoS ONE 7(4:e33544.doi:10.1371/journal.pone.0033544. According to Prof. Seno, mouse iPS cells (miPS when cultured in the conditioned medium derived from cancer cell lines, differentiate into cancer stem cells (CSCs. While differentiating into CSCs, they do retain the potential to develop endothelial progenitor cells. Several questions arise here: 1.Are these miPS derived CSCs really pluripotent, even if the terminal differentiation destined to specific phenotypes? 2.Shouldn’t the Cancer Stem Cells be termed as cancer progenitor cells, as till date they are considered to be producing only cancer cells but not pluripotent to yield other types of normal tissues? The spin-offs could be infinite as the process of differentiation and de-differentiation happening due to trillions of signals and pathways, most still remaining not-so-well understood. A special mention should be made to Prof. Shinya Yamanaka as he has several sterling qualities to be a role-model for budding scientists. Apart from his passion for science, which made him shift his career from orthopedics to a cell biologist, his

  3. Citizens Integrity Pledge ######################### I believe

    Indian Academy of Sciences (India)

    Administrator

    I believe that all stakeholders such as governrnent , citizens and the private sector need to work together to eradicate corruption. I realise that ... maintaining highest standards of integrity, transparency and good governance in all aspects of our.

  4. Teknologia Elektrikoa I. Laborategiko praktikak

    OpenAIRE

    Etxegarai Madina, Agurtzane; Larrabe Uribe, Zigor

    2012-01-01

    Helburuak: Teknologia Elektrikoa I ikasgaiko edukin teorikoei buruzko laborategiko praktikak burutzea. Norentzat: Teknologia Elektrikoa I ikasgaiko ikasleentzat. Praktika hauetan hurrengo softwarea erabiliko da: -1. Matlab/Simulink/SimPowerSystems -2. Mathcad -3. Powerworld -4. DigSilent

  5. How Can I Stop Cutting?

    Science.gov (United States)

    ... Educators Search English Español How Can I Stop Cutting? KidsHealth / For Teens / How Can I Stop Cutting? ... in a soft, cozy blanket Substitutes for the Cutting Sensation You'll notice that all the tips ...

  6. iMovie '09 & iDVD pocket genius

    CERN Document Server

    Hart-Davis, Guy

    2010-01-01

    If you want to get the very most out of iMovie '09 or iDVD, put this savvy Portable Genius to work. Want to quickly turn raw footage into a polished movie? Crop, rotate, or delete clips? Add background music or sound effects? Customize your iDVD themes? You'll find cool and useful Genius tips, insider secrets, full-color screenshots, and pages of easy-to-access shortcuts and tools that will save you loads of time and let you enjoy iMovie '09 and iDVD to the max.

  7. NATO Reference Mobility Model. Edition I. Users Guide. Volume I

    Science.gov (United States)

    1979-10-01

    C TOTAL ERAKING FCRCE - SCIL /SLOPE/VEHICLE C --- ----.----------- C 275 R-2WJ58# VCLUIJE I PAGE A-103 APPEND-IX A - LISTING CF O.CFAt’ NRtPM C 1...sGCW vN1RAV ,SFTYPC 9TBF I- c r-------------------------~e e C tPAXIMUP BRAKING FCRCE * SCIL /SL OP E/V ENICLE/ DRIVER C --------------------- C C 1. VAR...CONTINUE GO TC 2160 ,ýObO IFl IST ,NE, 21 GC TG 2110i 317 R-20358, VOLUME I PAGE A-145 APPENDIX A - LISTING CF FRGG/iAM NAMM C 8. COARSE GRAINED SCIL IF

  8. Imenovanje jednonitnih homopolimera i kopolimera na osnovi podrijetla (I. dio

    Directory of Open Access Journals (Sweden)

    Vida Jarm

    2018-04-01

    Full Text Available Prethodne IUPAC-ove preporuke imenovanja (nomenklature jednonitnih polimera na osnovi podrijetla (NOP odnosile su se uglavnom na kopolimere, nelinearne polimere, združene polimere i generičke polimere. Pravila navedena u ovim preporukama omogućuju jasnije i preciznije imenovanje polimera na osnovi podrijetla, kako homopolimera tako i kopolimera. Prikazani sveobuhvatni sustav imenovanja polimera na osnovi podrijetla prihvatljiva je alternativa sustavu imenovanja polimera na osnovi strukture. Zbog raširene i česte uporabe dodatno su opisane i preporuke za uporabu uvriježenih imena polimera.

  9. Induced QCD I: theory

    Energy Technology Data Exchange (ETDEWEB)

    Brandt, Bastian B. [Institute for Theoretical Physics, Goethe-University of Frankfurt,60438 Frankfurt (Germany); Institute for Theoretical Physics, University of Regensburg,93040 Regensburg (Germany); Lohmayer, Robert; Wettig, Tilo [Institute for Theoretical Physics, University of Regensburg,93040 Regensburg (Germany)

    2016-11-14

    We explore an alternative discretization of continuum SU(N{sub c}) Yang-Mills theory on a Euclidean spacetime lattice, originally introduced by Budzcies and Zirnbauer. In this discretization the self-interactions of the gauge field are induced by a path integral over N{sub b} auxiliary boson fields, which are coupled linearly to the gauge field. The main progress compared to earlier approaches is that N{sub b} can be as small as N{sub c}. In the present paper we (i) extend the proof that the continuum limit of the new discretization reproduces Yang-Mills theory in two dimensions from gauge group U(N{sub c}) to SU(N{sub c}), (ii) derive refined bounds on N{sub b} for non-integer values, and (iii) perform a perturbative calculation to match the bare parameter of the induced gauge theory to the standard lattice coupling. In follow-up papers we will present numerical evidence in support of the conjecture that the induced gauge theory reproduces Yang-Mills theory also in three and four dimensions, and explore the possibility to integrate out the gauge fields to arrive at a dual formulation of lattice QCD.

  10. Dark matter detection - I

    International Nuclear Information System (INIS)

    Zacek, Viktor

    2015-01-01

    The quest for the mysterious missing mass of the universe has become one of the big challenges of today's particle physics and cosmology. Astronomical observations show that only 1% of the matter of the universe is luminous. Moreover there is now convincing evidence that 85% of all gravitationally observable matter in the universe is of a new exotic kind, different from the 'ordinary' matter surrounding us. In a series of three lectures we discuss past, recent and future efforts made world-wide to detect and/or decipher the nature of Dark Matter. In Lecture I we review our present knowledge of the Dark Matter content of the Universe and how experimenters search for it's candidates; In Lecture II we discuss so-called 'direct detection' techniques which allow to search for scattering of galactic dark matter particles with detectors in deep-underground laboratories; we discuss the interpretation of experimental results and the challenges posed by different backgrounds; In Lecture III we take a look at the 'indirect detection' of the annihilation of dark matter candidates in astrophysical objects, such as our sun or the center of the Milky Way; In addition we will have a look at efforts to produce Dark Matter particles directly at accelerators and we shall close with a look at alternative nonparticle searches and future prospects. (author)

  11. iCSC2010

    CERN Multimedia

    IT Department

    2010-01-01

    The 4th edition of the Inverted CERN School of Computing (iCSC, “Where students turn into teachers”) will take place next Monday and Tuesday (8th and 9th of March) at CERN, Building 31 - IT Amphitheatre - Third Floor. Attendance is free and open to everyone. The programme is designed so that you can attend the lectures that interest you. Registration is not mandatory, but will allow you to obtain a copy of the full booklet (first registered, first served). Programme overview: Monday 8 March 10:00 - 10:15 Introduction School opening 10:15 - 11:10 Lecture 1 Introduction to UML for Developers and OO Best Practices - Tim Muenchen 11:20 - 12:15 Lecture 2 Design Patterns and Anti-Patterns - Tim Muenchen 14:00 - 14:55 Lecture 3 How to make web applications run faster - David Horat 15:05 - 16:00 Lecture 4 Distributed version control using Git: Get more efficient managing your code - David Svantesson 16:30- 17:25 Lecture 5 Recent Advances in Multivariate Data Visualisation - Benjam...

  12. Administration i politisk styrede organisationer

    DEFF Research Database (Denmark)

    Hansen, Steen Juul

    2017-01-01

    At redegøre for hvad administration i politisk styrede organisationer er og forklare relevante teorier og begreber, der kan bruges til at analysere og forstå administrativt arbejde i politisk styrede organisationer.......At redegøre for hvad administration i politisk styrede organisationer er og forklare relevante teorier og begreber, der kan bruges til at analysere og forstå administrativt arbejde i politisk styrede organisationer....

  13. Et lille hus i byen

    DEFF Research Database (Denmark)

    . Bebyggelserne, der blev opført som billige arbejderkvarterer, er i dag eftertragtede enklaver for velstillede københavnere. Populariteten bunder ikke alene husenes historie, men også i en række andre forhold, som arkitekter og planlæggere kan lære af i dag. I en tid med et stort behov for billige boliger, er...

  14. Ensretning i den internationale skatteret

    DEFF Research Database (Denmark)

    Schmidt, Peter Koerver; Bundgaard, Jakob

    2015-01-01

    I lyset af den seneste tids begivenheder (herunder særligt Luxleaks-sagerne) og tiltag på den internationale skattescene ses der i artiklen nærmere på en række af de forhold, som trækker i retning af øget ensretning af selskabsskattereglerne i store dele af verden. Det gøres gældende, at staternes...

  15. POJAM I STRUKTURA SOCIJALNOG SUKOBA

    OpenAIRE

    Vlah, Nataša

    2010-01-01

    Autorica polazi od pretpostavke da je profesionalcima u odgojnom i obrazovnom radu danas neophodno razumijevanje socijalnih sukoba jer je učinkovitost u upravljanju socijalnim sukobima prediktor dobrih međuljudskih odnosa u vrtićima, školama, domovima i drugim odgojno-obrazovnim ustanovama. Takvi su odnosi poticajno okruženje optimalnih razvojnih ishoda za pojedino dijete i adolescenta. Cilj rada je dati teorijsku analizu pojma i strukture socijalnih sukoba sa svrhom boljeg razumijevanj...

  16. Inner ionization in A sup I sup I B sup V sup I

    CERN Document Server

    Komashchenko, A V; Kolezhuk, K V; Sheremetova, G I; Fursenko, V D; Bobrenko, Y N

    2002-01-01

    The dependences of the sensitivity of the p-Cu sub 1 sub . sub 8 S/n-A sup I sup I B sup V sup I -type surface-barrier heterostructures on the energy of exciting photons or accelerated monoenergetic electron beams are investigated. A technique for determination of the mean internal ionization energy epsilon in direct-gap A sup I sup I B sup V sup I compounds is suggested and epsilon values are found experimentally. It is shown that the relationship between epsilon and the semiconductor energy gap E sub g is given by the following expression epsilon 2.5E sub g

  17. Nye ledelsesudfordringer i forebyggelse af stress i videnarbejdet

    DEFF Research Database (Denmark)

    Andersen, Vibeke; Ipsen, Christine; Mogensen, Mette

    arbejdssituation og muligheder for forebyggelse af arbejdsrelateret stress i vidensarbejdet. – Et kvalitaivt studie af arbejdsrelateret stress i vidensarbejdet. Lyngby, The Technical University of Denmark. Kärreman, Dan, Stefan Svenningson and Mats Alvesson (2003): The Return of the Machine Bureaucracy? Management......Afsættet for projektet ”Forebyggelse af stress i videnarbejdet – mellem begejstring og belastning” er, at der er behov for en ny forståelse af stress i det moderne arbejdsliv, for at kunne arbejde med stressforebyggelse blandt videnarbejdere. I dette paper tager vi udgangspunkt i begrebet...... mangetydighed/ambiguity som en karakteristik af videnarbejdet, da det stiller nogle helt nye udfordringer til hvordan ledelse af videnarbejdet skal foregå, når målet er at forebygge arbejdsrelateret stress. Vi karakteriserer videnarbejdet som mangetydigt og som Alvesson (2004) påpeger, udgør dilemmaerne i...

  18. Der er liv i historie

    DEFF Research Database (Denmark)

    Poder, Anette; Müller Glibstrup, Tine; Roj Søndergaard, Anja

    I denne antologi præsenteres de første konkrete bud på, hvordan en senmoderne og konstruktivistisk historieundervisning kan tage sig ud i praksis. Der tages udgangspunk i bl.a. Bernard Eric Jensen "Historie - livsverden og fag" og Carlo Grevy "Historie og livsverden - en historiedidaktik"...

  19. Arbejdsgiver-organisationer i Danmark

    DEFF Research Database (Denmark)

    Navrbjerg, Steen Erik; Ibsen, Christian Lyhne

    Den moderne arbejdsgiverorganisation er både arbejdsgiverorganisation og brancheorganisation – og har formået at tilpasse sig i en tid, hvor medlemmerne ændrer profil under pres fra skarp international konkurrence, ny teknologi og fald i lønmodtagerorganiseringen. Det er et af resultaterne i en F...

  20. PISA - komparativ evaluering i storskalaformat

    DEFF Research Database (Denmark)

    Dolin, Jens

    2008-01-01

    Internationale evalueringer som PISA sætter i stadig højere grad dagsordenen i den uddannelsespolitiske debat og har stor indflydelse på deltagerlandenes uddannelsespolitiske beslutninger. Men berettiger undersøgelserne til den ståhej, de ofte afstedkommer? Artiklen vil gå ind i krydsfeltet melle...

  1. Kirken og pesten i senmiddelalderen

    DEFF Research Database (Denmark)

    Bisgaard, Lars

    2008-01-01

      De danske pestepidemier i senmiddelalderen er aldrig tidligere blevet behandlet. For det første har de stået helt i skyggen af den første pestbølge, som allerede i den tidlige historieskrivning om pesten blev et samlende symbol for de kvaler, befolkningen udstod. For det andet har forskningen s...

  2. Location i det geopolitiske rum

    DEFF Research Database (Denmark)

    Agger, Gunhild

    2017-01-01

    Hovedformålet med artiklen er at undersøge forholdet mellem genrehåndtering og location i The Night Manager. Genren er thrilleren i den spionudgave, der er kendt fra tidligere John Le Carré-filmatiseringer. Location passer perfekt til genren med valget af glamourøse steder, der har kunnet bruges i...

  3. iPads og undervisningsdifferentiering

    DEFF Research Database (Denmark)

    Lorentzen, Rasmus Fink

    2013-01-01

    Artiklen er blevet til på baggrund af udviklingsprojektet med iPad i Odder Kommune. Den generaliserer på baggrund af en række eksempler herfra og peger på at med iPad'en drejer det sig om at udnytte mulighederne for at lade eleverne arbejde producerende, kollaborativt og fagligt meningsfuldt...

  4. Kulturel liminalitet i Don Quixote

    DEFF Research Database (Denmark)

    Fastrup, Anne

    2007-01-01

    Med udgangspunkt i og i kontrast til nogle af hovedlinierne i den nyere litteratur om Don Quixote søger artiklen tilbage til de mere specifikke og lokale historiske forhold, som er romanens baggrund, bl.a. til Cervantes' »algierske erfaring« med tyrkisk fangenskab. Artiklen forsøger at kombinere ...

  5. Quark i mattoni del mondo

    CERN Document Server

    Fritzsch, Harald

    1983-01-01

    Quark rossi, verdi e blu ; quark dotati di stranezza e di incanto ; quark 'su' e 'giù' : sembra che i fisici delle particelle giochino a confondere la curiosità del profano, con queste denominazioni fantasiose. Che cosa significano ? e, soprattutto, i quark sono i costituenti davvero elementari della materia ?

  6. Ledelse af forandringer i folkeskolen

    DEFF Research Database (Denmark)

    Kjer, Mikkel; Rosdahl, Anders

    2016-01-01

    Dette notat bygger på kvalitative interview på seks folkeskoler i efteråret 2015. Skolerne ligger i forskellige egne af landet og i landområder, mindre og store byer. Skolerne er af forskellig størrelse, og deres elevgrundlag, dvs. forældrenes sociale baggrund, varierer også. Notatet bygger primæ...

  7. Benchmarking i den offentlige sektor

    DEFF Research Database (Denmark)

    Bukh, Per Nikolaj; Dietrichson, Lars; Sandalgaard, Niels

    2008-01-01

    I artiklen vil vi kort diskutere behovet for benchmarking i fraværet af traditionelle markedsmekanismer. Herefter vil vi nærmere redegøre for, hvad benchmarking er med udgangspunkt i fire forskellige anvendelser af benchmarking. Regulering af forsyningsvirksomheder vil blive behandlet, hvorefter...

  8. Olie skaber fred i Sudan

    DEFF Research Database (Denmark)

    Laugesen, Henrik

    Sydsudan forventes formelt at erklære sin uafhængighed den 9. juli 2011. Men hvad sker der, når Sudan nu deles i Nordsudan og Sydsudan? Oliekilderne ligger i Sydsudan, men den helt nødvendige infrastruktur der skal transformere olie til dollars har man i nord. Da op imod 90% af Sydsudans indtægter...

  9. Rural Utopi i Urban Tidsalder

    DEFF Research Database (Denmark)

    Bagger-Petersen, Mai Corlin

    2015-01-01

    Den kinesiske filmskaber, redaktør, kurator og kunstner Ou Ning udformede i 2010 notesbogen Hvordan man laver sin egen utopi; en forestilling om en intellektuel genoplivning af et tabt, ruralt fællesskab i landsbyen Bishan i den østlige del af Centralkina. Notesbogen er et montageværk, som afføde...

  10. Videoer om feedback i undervisningen

    DEFF Research Database (Denmark)

    Jensen, Hanne Nexø

    2017-01-01

    I denne video bliver du introduceret til en måde at praktisere og rammesætte klyngevejledning på i bachelorundervisning. Klyngefeedbackformen til de studerende er valgt, da de studerende lærer meget af både at give og om modtage feedback fra medstuderende. Fokus på feedback ligger derfor primært i...

  11. Professionaliseringen i den offentlige sektor

    DEFF Research Database (Denmark)

    Hjort, Katrin

    fokuserer bl.a. på CVU-dannelser og nye professionsbacheloruddannelser, men også på de generelle ændringer, der finder sted i relationsarbejdets organisering (A-/B-hold), i de arbejdsmæssige relationer til klienter/brugere/kunder og i arbejdets vidensfundament (udviklingen af nye styringsvidenskaber)....

  12. I Will Resist Being Labelled

    DEFF Research Database (Denmark)

    Walther-Hansen, Mads

    2015-01-01

    Det sidste i vores serie af professorportrætter er af Mark Grimshaw, der siden 2012 har været professor i musik ved Aalborg Universitet. Han er forskningsleder for vidensgruppen MaSK (Music and Sound Knowledge Group), hvor han bl.a. beskæftiger sig med biofeedback teknologier i lyddesign til...

  13. SPORT I POLITIKA

    Directory of Open Access Journals (Sweden)

    Mensur Memić

    2005-04-01

    Full Text Available Sport and politic are two big appearance and human activities which have as difference so the same segments. New society, democratic society necessity cultivate realization of sport-politic.We could say they are dominates phenomens of modern society. Like this they are manifestationed on the different necessity ways and have different roles. They are sa different as same. Everybody who take part in one or other sphere know from society from sociolog aspect how are two society components connected and reservationed beetwen. Why I am telling it: because the future of sportsmen depend from the future of politics will. Its needed to show how politic manage or give s the hope for development and animate possibility that young people during their evolution can do sport. Politic also depend from sports instutitions on creation mood of young people that their cadres got general and society dope. These two concepts can be near define. Sport as activity and as a appeal represent specific sights orientation of young people in aim curing knight games and evervthing in aim of health formation and effort of results, but they are not only meanings, through the sport can progress and big number acquaintance, friendship and intimacy, through sport ali cultural heredity can be keeping and the lands vvhich belong. Sport sociological looking is important element society life people. Second concept-politic, according to the famous politicians we can definite as a action that direct with another activities and business. Politic is substance, process of manifestation, manner, and it mean that politic has its subject activities vvhich is consist on behavour other social groups.

  14. Petroleum resources assessment (I)

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1998-12-01

    This report consists of 2 subjects. 1) Petroleum resources assessment of the Kunsan Basin : Kunsan Basin is mainly filled with Cretaceous and Tertiary clastic sediments, and divided into Southwest Sub-basin, Central Sub-basin and Northeast Sub-basin by uplifts and faults developed in the basin. Microfossils were studied for the biostratigraphic works of drill wells in the Kunsan Basin. The microfossils include organic-walled microfossils such as spores, pollen and nonmarine dinoflagellates and calcareous microfossils such as ostracods, charophytes and gastropods. The fossil assemblages of the Kunsan Basin reveal nonmarine environments ranging from alluvial fan to shallow lacustrine and climatic variation between subtropical and cool temperate temperature in the arid/humid alternating conditions. According to the paleontological data, the Kunsan Basin was initiated in the Early Cretaceous and expanded during Paleogene followed by regional erosion at the closing time of Paleogene on which Neogene sediments have been accumulated. The Paleogene strata show laterally irregular thickness in each Epoch due to migrating depocenter. 2) Petroliferous basin analysis in Hapcheon area (I) : The Cretaceous Gyeongsang Supergroup consists of more than 9 Km sequences of sedimentary and volcanic rocks in Hapcheon-Changyong-Euiryong-Haman area and occupies the middle part of the Milyang subbasin. The Supergroup can be divided into three group; Sindong, Hayang and Yuchon groups in ascending order. Based on rock color, the Sindong Group can be subdivided into Nakdong, Hasandong and Jinju Formations. The Hayang Group can be subdivided into Chilgok, Silla Conglomerate, Haman and Jindong Formations. The Chilgok Formation includes basaltic lava and tuffs in the upper part. The Haman Formation has Kusandong tuff (keybed) in the uppermost part in the Changyong area, whereas the tuff is intercalated below the vocaniclastics in the Haman area. (author). 60 refs., 22 tabs., 61 figs.

  15. STROJEVI ZA PRIJENOS I DIZANJE

    OpenAIRE

    Mesić, Slaven

    2016-01-01

    U ovom završnom radu obrađeni su strojevi za prijenos i dizanje materijala. Strojevi za prijenos i dizanje su posebni strojevi namijenjeni unutarnjem (gradilišnom) transportu. Prikazane su opće podjele i karakteristike toranjskih dizalica, mobilnih dizalica, kabel kranova, „Derrick kranova“, mosnih kranova i građevinskih dizala. Uporaba strojeva za prijenos i dizanje je uvjetovana masom tereta koji je potrebno prenijeti te specifičnim uvjetima na gradilištu. Odabir potrebnog st...

  16. Antoni Pous, poeta i traductor

    OpenAIRE

    Farrés Puntí, Ramon

    2002-01-01

    Consultable des del TDX Títol obtingut de la portada digitalitzada Antoni Pous, poeta i traductor és un estudi monogràfic de la figura d'Antoni Pous i Argila (Manlleu, 1932 - Barcelona, 1976), activista cultural i home de lletres que va destacar de molt jove, en la immediata postguerra, com a poeta de gran talent; que als anys seixanta va dur a terme una tasca cultural i pedagògica de gran transcendència a Igualada, amb la fundació del grup Lacetània i la creació de la revista Textos; q...

  17. Origin of I in soil and the 129I problem

    International Nuclear Information System (INIS)

    Cohen, B.L.

    1985-01-01

    It is widely believed that the I in soil is derived from the I in oceans by evaporating-atmospheric transport-washout by rain over land, rather than from the I in rock when the latter is weathered into soil. Since human I burdens are derived largely from soil by plant root uptake into food, this implies that the specific activity of 129 I in the I of human thyroids will be the same as that in the oceans, whereas it would be similar to that in rock (much lower) if the I in soil were derived from weathering of rock as is the case for other heavy trace elements. The bases for the oceanic origin theory are analyzed and found not to be in accord with modern data. Recent data shows no correlation between concentration of I in soil and proximity to oceans, which argues strongly against the oceanic origin theory. Some problems with long-distance atmospheric transport are pointed out. It is concluded that only a small fraction of the I in soils is of oceanic origin. Most estimates of very long-term effects of 129 I on human health are therefore several times too high

  18. First evidence of [i]Babesia venatorum[/i] and [i]Babesia capreoli[/i] in questing Ixodes ricinus ticks in the Czech Republic

    Directory of Open Access Journals (Sweden)

    Kristyna Venclikova

    2015-05-01

    Full Text Available Introduction and objective. [i]Ixodes ricinus[/i] is the most common tick species occurring in Central Europe and it serves as a principal vector of emerging human pathogens. The aim of this study was to determine the prevalence of [i]Babesia spp[/i]. in host-seeking [i]I. ricinus[/i] in urban and natural habitats. Materials and methods. PCR was applied on samples to assess prevalence of [i]Babesia spp.[/i] in questing ixodid ticks. Sequencing was used for [i]Babesia[/i] species determination. Results. 1,473 [i]I. ricinus[/i] ticks (1,294 nymphs, 99 males and 80 females were examined for the presence of [i]Babesia spp[/i]. at the two study sites. Minimum infection rate for [i]Babesia[/i] spp. was found to be 0.5% (infected I. ricinus nymphs were only detected in the natural ecosystem. Two[i] Babesia[/i] species were identified by sequencing: [i]B. venatorum[/i] (formerly called[i] Babesia[/i] sp. EU1 and [i]B. capreoli. [/i] Conclusions. The results obtained represent the first evidence of the occurrence of [i]B. venatorum[/i] and [i]B. capreoli[/i] in host-seeking[i] I. ricinus[/i] ticks in the Czech Republic.

  19. I believe in Sherlock Holmes

    DEFF Research Database (Denmark)

    Lauridsen, Palle Schantz

    2012-01-01

    Sherlock Holmes så første gang dagens lys i 1887. I 4 romaner og 56 korte fortællinger berettede Arthur Conan Doyle i en periode på 40 år om de kriminalsager, hans consulting detective løste i samarbejde med sin ven, hjælper og kronikør, Dr. John Watson. Allerede, mens Doyle stadig skrev om Holmes......, vandrede hans figur over i andre medier, og historierne om ham hører i dag til de mest adapterede i verden. Inden for de sidste år er Holmes igen blevet populær både på film og tv. Både BBC’s tv-serie og Guy Ritchies film forholder sig legende til Doyles univers og til Holmes’ seksualitet....

  20. iPad Made Simple

    CERN Document Server

    Trautschold, Martin; Learning, MSL Made Simple

    2010-01-01

    The new iPad is sleek, powerful, and most importantly, it's much more than just a big iPhone. Your iPad is can be used for reading, surfing the web, emailing, watching TV/Movies, getting work done, and much more. And with the upcoming wave of iPad apps, the possibilities are endless. iPad X Made Simple clarifies all of the key features on the iPad, introduces what's new, and also reveals dozens of time-saving shortcuts and techniques. The book has over 1,000 screen shots that are carefully annotated with step-by-step instructions. * Clear instructions on how to set up and use the iPad * Illust

  1. S. S. Chern and I

    Science.gov (United States)

    Yang, Chen Ning

    2013-05-01

    I do not remember whether I had met Prof. S. S. Chern when he was a graduate student of Tsinghua University in Peking (now Beijing) where my father was a mathematics professor, and I was in elementary school. But I do remember how I had met Mrs. Chern for the first time, in early October, 1929, when I was seven years old and she was in junior high school. Her father, Professor Tsen, had been a professor of Mathematics at Tsinghua University already for a number of years, and the Yangs were new comers that fall. The Tsens invited us to their house for dinner and that was when I first made the acquaintence of "big sister Tsen"...

  2. A Survey of Mucilaginous Testa in <i>Chamaesyce>

    Directory of Open Access Journals (Sweden)

    Jordan, M. S.

    1992-12-01

    Full Text Available Seeds of <i>Chamaesyce> were studied for presence of mucilaginous testa. Samples were selected to represent all major systematic sections within the genus. Observations were made with a dissecting microscope following brief hydration of seeds; additional SEM observations of both intact and fractured dry seeds were made for selected species. With few exceptions, most of Boissier's subsections test positively fo r seed mucilage; however, mucilage is generally absent in subsections <i>Gymnadeniae> and "<i>Sclerophyllae>", for wich absence of mucilage is regarded as secondary loss from mucilagionus ancestors. Although mucilage production is associated with a well-defined subepidermal layer of macroesclereids, it is the surface layer of cells that actually secrete mucilage. Mucilage secreting cells and macroesclereids of mature testa are interpreted to develop from the epidermal layers of outer and inner integuments, respectively. Seed mucilages may play a role in seed hydration and/or seed dispersal.

    Han estat estudiades les granes de <i>Chamaesyce> segons la presència o no de testa mucilaginosa. Les mostres van ésser seleccionades per tal de representar tot es les principals seccions del gènere. Les observacions han estat fetes al microscopi de disseció desprès d'hidratar breument les llavors; addicionalment s'han fet observacions en el MER de llavors seques, senceres i fracturades , de les espècies seleccionades. Amb poques excepcions, la major part de les subseccions de Boissier presenten els tests de presència de mucílags a les granes positius; no obstant, els mucilags generalment no es presenten en les subseccions <i>Gymnadeniae> i "<i>Scterophyltae>", per a les quals l'absència de mucilags es considerada comuna pèrdua secundària a partir d'avantpassats mucilaginosos. Encara que la producció de mucilags s'associa amb les capes subepidèrmiques ben definides de les macroesclereides, en realitat es la capa de

  3. Taxonomic and ecological review of California <i>Delphinium>

    Directory of Open Access Journals (Sweden)

    Warnock, Michael J.

    1990-12-01

    Full Text Available Included within the boundaries of California are all or parts of the ranges of many of the <i>Delphinium> native to North America.Currently, 28 species of <i>Delphinium> are recognized as occurring in California. In addition to nominal subspecies in these species. another 17 subspecies are recognized. A key to, and descriptions of, the taxa of <i>Delphinium> found in California are presented. 1ncludedwith descriptions are comments on distribution, habitat preferences, propensity for hybridization and abundance of each taxon. Certain hybrids which may be found (often as a result of human disturbance are separated in the key.

    [ca] A l'interior dels límits de Califòrnia es troben la totalitat o parts de les àrees de bona part dels <i>Delphinium> nadius de Nordamèrica. Es reconeixen 28 espècies de <i>Delphinium> a Califòrnia; ultra llurs respectives subspècies nominals, es reconeixen 17 altres subspècies. Es presenten un a clau i les descripcions corresponents als tàxons presents a Califòrnia. Juntament amb les descripcions, s'hi inclouen comentaris sobre la distribució, preferències d'hàbitat, propensió per a ta hibridació i abundància de cada tàxon. Alguns híbrids que es poden trobar (sovint com a resultat de pertorbacions causades per l'home són separats a les claus.

  4. Presencia de <i>Lactobacillus sppi>. y <i>Bacillus licheniformisi> en margarina con yogur

    Directory of Open Access Journals (Sweden)

    González, Elisa

    1998-02-01

    Full Text Available The microbiological analysis of thirty samples of commercially produced margarine with incorporated yoghurt was carried out. After the initial control, the other tests were runned after 26, 56, 88,116 and 157 days of refrigerated storage. Constitutive biota of yoghurt was not detected. Occurrence (100% of samples of <i>Lactobacillus spp.i> (<i>Lactobacillus fermentumi> and <i>Lactobacillus caseii> subsp. <i>pseudoplantarum>, being the first one slightly more numerous, and <i>Bacillus licheniformisi>, which counts were mostly in a 103- 104ufc/g range, and only in 10% of the cases were < 103 ufc/g. Samples did not show signs of deterioration. Article calls upon about the convenience of developing a specific normative that clarifies the doubts about main microbiological hazards as well as the legal aspects regarding the product denomination.

    Se ha realizado el análisis de treinta muestras de margarina con yogur. Tras el control inicial, los restantes análisis se efectuaron a los 0,26, 56, 88,116 y 157 días de almacenamiento en refrigeración. No se detectó la biota constitutiva del yogur. Sí se demostró la presencia, en el 100% de las muestras, de <i>Lactobacillus fermentumi> y <i>Lactobacillus caseii> subsp. <i>pseudoplantarum>, siendo el primero ligeramente más numeroso, así como de <i>Bacillus licheniformisi>, cuyos recuentos han sido en su mayoría comprendidos en el rango 103-104ufc/g, y sólo en el 10% de los casos fueron inferiores a 103ufc/g. Las muestras no mostraban signos de deterioro. El trabajo llama la atención sobre la conveniencia de desarrollar una normativa específica que aclare las dudas surgidas en torno a los principales riesgos microbiológicos, así como a aspectos legales relacionados con la denominación del producto.

  5. Summary of current radiation dose estimates to humans from 123I, 124I, 126I, 130I, and 131I as sodium rose bengal

    International Nuclear Information System (INIS)

    Anon.

    1975-01-01

    Estimated absorbed doses to human gall bladder, gastrointestinal tract, liver, ovaries, bone marrow, and testes from 123 I, 124 I, 126 I, 130 I, and 131 I after a single intravenous administration as sodium rose bengal are summarized. The greatest uncertainty in these dose estimates is due to the variability in time for the movement of radioiodine through the biliary tract, gall bladder, and gastrointestinal tract

  6. 123I and 13I purification for biomolecules labelling

    International Nuclear Information System (INIS)

    Catanoso, Marcela Forli

    2011-01-01

    The 123 I and 131 I are iodine radioisotopes widely used in Nuclear Medicine. The radioisotope 123 I is used in diagnosis through the SPECT technique and is routinely produced at IPEN in cyclotron through the reaction: '1 24 Xe (p, 2n) '1 23 Cs -> 123 Xe -> 123 I. The radioisotope 131 I is used both in diagnosis and therapy due to its physical characteristics of decay by β - and its γ-ray emissions that are softened with the use of specific collimators for diagnosis. It is routinely produced at IPEN using the nuclear reactor through the indirect reaction: 130 Te (n, γ) -> 131 Te -> 131 I, irradiating compounds containing Te. The radiopharmaceuticals prepared with these radioisotopes go through rigorous quality control tests and the chemical purity of the primary radioisotopes 123 I and 131 I are within the permissible limits currently defined. However, the presence of some chemical contaminants can prejudice the biomolecules labeling (monoclonal antibodies and peptides), that will produce radiopharmaceuticals of first generation to the oncology area. The aim of this work was to obtain a new purification method of these radioisotopes, allowing the labeling of biomolecules and also to established a process control on those radioisotopes. The methodology was separated on 3 steps: Evaluation of '1 23 I e 131 I radionuclidic purity using a hyper pure germanium detector, chemical purity using ICP-OES and the retention and elution study of 131 I in several absorbers to choose the most appropriate for the purification tests analyzing the behavior of the possible contaminants. The radionuclidic analyses showed the presence of Te and Co on 131 I samples and Te, Tc e Co on 123 I samples. The chemical purity analyses showed the presence of Al and Mo in 123 I, coming from the window material of the target holder and the presence of Al and Te in 131 I samples, coming from the target holder and the target, respectively. The retention and elution study selected the most

  7. iOS 6 foundations

    CERN Document Server

    Feiler, Jesse

    2013-01-01

    A practical introduction for using iOS 6 to create universal apps If you have prior experience programming in an object-oriented language and are eager to start building universal apps for iPad and iPhone (including the iPod touch), then this is the book for you! Using the latest version of iOS (iOS 6) along with the latest version of Xcode (Xcode 4.5), this book is a practical introduction rather than just a catalog of components. Full-color and packed with groundbreaking, innovative designs, this book teaches you how to create eye-catching, unique apps. Teaches you the va

  8. iPhone For Dummies

    CERN Document Server

    Baig, Edward C

    2010-01-01

    The full-color guide to getting the most out of your iPhone. Completely updated and revised throughout, this full-color guide covers Apple's new iPhone and iOS 4. Bestselling veteran authors Baig and LeVitus introduce you to the capabilities of the iPhone whether you're making phone calls, browsing the Internet, sending and receiving e-mails, working with the calendar, watching videos, taking great photos, or much more. You'll discover how to set up iTunes, buy music and videos from the iTunes store, protect your information, troubleshoot, multitask, and download the hundreds of thousands of a

  9. Rafael Tasis i el teatre

    OpenAIRE

    Foguet i Boreu, Francesc

    2013-01-01

    En aquest article deixem de banda al dedicació de Tasis a la traducció teatral, que ja ha estat analitzada per Sílvia-Coll-Vinent en el seu estudi i que, en canvi, esmenta Josep Poblet (1966:106): "El Senyoret Dudley", de Philip Jhonson i "Romanoff i Julieta" de Peter Ustinov, unes peces que li foren encarregades explícitament "amb la promesa d'una immediata estrena", que no es produí mai.

  10. Developing iPhone application

    OpenAIRE

    Nebo, Charles

    2015-01-01

    IPhone application popularity and demand are continuously on the increase since 2008 when Apple launched iPhone. Consequently, many applications are built targeting different user needs. But developing iPhone applications do have challenges. This research study explores current development study for developing iPhone application where the development environment and technologies are problematic. This paper intends to review some software development methodologies, methods, and techniques for ...

  11. "I Did It My Way"?

    DEFF Research Database (Denmark)

    Jacobsen, Michael Hviid; Ferrari Wanseele, Janet

    2009-01-01

      I denne artikel beskrives de senere års forandringer i dele af den danske døds- og mindekultur med fokus på, hvorledes den enten adskiller sig fra eller skal ses som en forlængelse af den i samfundsforskningen ofte fremhævede fortrængte og tabuiserede dødskultur fra det 20. århundrede. Gennem a...

  12. Borger i det moderne Babel

    DEFF Research Database (Denmark)

    Delman, Thomas Fabian

    2011-01-01

    Hvordan agerer borgerne i den grænseløse by? Er vi blevet borgere i et moderne Babel, hvor vi ikke længere taler samme sprog? Hvorfor vil vi ikke have de fælles projekter i vores baghave? Og kan nyaktivisme måske være en mulig vej frem? Artiklen forsøger at give svar på nogle af disse spørgsmål....

  13. Nye sociale teknologier i folkeskolen

    DEFF Research Database (Denmark)

    deres selvstændighed og myndighed, så godt som de kunne. Prøveformen har bredt sig alvorligt ud i skolens almindelige hverdag, og hvis eleverne trænes i test, efterlader det ikke så meget plads til fortolkning, analyse og vurdering. Vi skal ikke gå i panik, men vi skal tænke os om," siger Lejf Moos...

  14. Vitamin D receptor gene Alw I, Fok I, Apa I, and Taq I polymorphisms in patients with urinary stone.

    Science.gov (United States)

    Seo, Ill Young; Kang, In-Hong; Chae, Soo-Cheon; Park, Seung Chol; Lee, Young-Jin; Yang, Yun Sik; Ryu, Soo Bang; Rim, Joung Sik

    2010-04-01

    To evaluate vitamin D receptor (VDR) gene polymorphisms in Korean patients so as to identify the candidate genes associated with urinary stones. Urinary stones are a multifactorial disease that includes various genetic factors. A normal control group of 535 healthy subjects and 278 patients with urinary stones was evaluated. Of 125 patients who presented stone samples, 102 had calcium stones on chemical analysis. The VDR gene Alw I, Fok I, Apa I, and Taq I polymorphisms were evaluated using the polymerase chain reaction-restriction fragment length polymorphism analysis. Allelic and genotypic frequencies were calculated to identify associations in both groups. The haplotype frequencies of the VDR gene polymorphisms for multiple loci were also determined. For the VDR gene Alw I, Fok I, Apa I, and Taq I polymorphisms, there was no statistically significant difference between the patients with urinary stones and the healthy controls. There was also no statistically significant difference between the patients with calcium stones and the healthy controls. A novel haplotype (Ht 4; CTTT) was identified in 13.5% of the patients with urinary stones and in 8.3% of the controls (P = .001). The haplotype frequencies were significantly different between the patients with calcium stones and the controls (P = .004). The VDR gene Alw I, Fok I, Apa I, and Taq I polymorphisms does not seem to be candidate genetic markers for urinary stones in Korean patients. However, 1 novel haplotype of the VDR gene polymorphisms for multiple loci might be a candidate genetic marker. Copyright 2010 Elsevier Inc. All rights reserved.

  15. Træernes rolle i et landbrugslandskab i Senegal

    DEFF Research Database (Denmark)

    Pedersen, Jens Christian

    2010-01-01

    Ved at gå ind i et eksisterende projektsamarbejde er det lykkedes Steen Christensen at gennemføre et vellykket feltarbejde i Senegal inden for et 6-måneders speciale. Resultaterne fra projektet vil kunne indgå som grundlag for en lokal forvaltning som tager sigte på at beskytte træerne som er en...

  16. Panel: If I Only Knew Then What I Know Now

    Science.gov (United States)

    Goals Recycling Green Purchasing Pollution Prevention Reusing Water Resources Environmental Management Discussion and Networking Event Panel: If I Only Knew Then What I Know Now WHEN: May 16, 2016 2:30 PM - 5:00 Alamos CONTACT: Veronika Mocko CATEGORY: Community INTERNAL: Calendar Login Event Description The LANL

  17. Modeling Li I and K I sensitivity to Pleiades activity

    NARCIS (Netherlands)

    Stuik, R.; Bruls, J.H.M.J.; Rutten, R.J.

    1996-01-01

    We compare schematic modeling of spots and plage on the surface of cool dwarfs with Pleiades data to assess effects of magnetic activity on the strengths of the L II and K I resonance lines in Pleiades spectra. Comprehensive L II and K I NLTE line formation computation is combined with comparatively

  18. Fra Jagannātha til <i>juggernaut>

    DEFF Research Database (Denmark)

    Aktor, Mikael

    2017-01-01

    Vognprocessionen i Puri, Odisha, er en af verdens største religiøse processioner. Omkring en million pilgrimme følger de tre gude-søskende, Jagannātha, Subhadrā og Balabhadra, hver i deres egen gigantiske processionsvogn trukket af mange hundrede pilgrimme, på deres rejse fra det store Jagannātha...

  19. Historie i spillefilm - for elever

    DEFF Research Database (Denmark)

    Tønnesen, Lone Guldbrandt

    2016-01-01

    MOOC om historiebrug i spillefilm, hvor elever kan lære om, hvordan spillefilm bruger fortiden, og om hvordan vores opfattelse af historien både sætter sig spor i filmene og bliver påvirket af dem.......MOOC om historiebrug i spillefilm, hvor elever kan lære om, hvordan spillefilm bruger fortiden, og om hvordan vores opfattelse af historien både sætter sig spor i filmene og bliver påvirket af dem....

  20. Hybrid Nanocomposite Photovoltaics, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — This Small Business Innovation Research Phase I project will develop an innovative solar cell design that combines nanotechnology with conducting polymer...