Sample records for mucopolysaccharidosis i
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Mucopolysaccharidosis type I: current knowledge on its pathophysiological mechanisms.
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.
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Orthopedic Pathology in Children with Mucopolysaccharidosis Type I
Directory of Open Access Journals (Sweden)
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.
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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
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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
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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.
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Diagnosing mucopolysaccharidosis IVA
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
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Phenotype prediction for mucopolysaccharidosis type I by in silico analysis.
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.
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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.
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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
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Cerebral magnetic resonance findings during enzyme replacement therapy in mucopolysaccharidosis
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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
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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.
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Correction of murine mucopolysaccharidosis VII by a human. beta. -glucuronidase transgene
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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.
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Directory of Open Access Journals (Sweden)
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.
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Features of Brain MRI in Dogs with Treated and Untreated Mucopolysaccharidosis Type I
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
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Directory of Open Access Journals (Sweden)
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
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Altered corneal stromal matrix organization is associated with mucopolysaccharidosis I, III and VI.
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.
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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.
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Directory of Open Access Journals (Sweden)
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.
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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.
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Directory of Open Access Journals (Sweden)
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.
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Directory of Open Access Journals (Sweden)
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.
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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.
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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.
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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.
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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.
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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...
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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
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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.
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Directory of Open Access Journals (Sweden)
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
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Directory of Open Access Journals (Sweden)
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.
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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
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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
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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
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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
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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
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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
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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
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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.
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Genetics Home Reference: mucopolysaccharidosis type I
... 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. ...
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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.
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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...
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Diagnostic and treatment strategies in mucopolysaccharidosis VI
Directory of Open Access Journals (Sweden)
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
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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.
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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
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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.
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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
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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
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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...
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Distribution of Heparan Sulfate Oligosaccharides in Murine Mucopolysaccharidosis Type IIIA
Directory of Open Access Journals (Sweden)
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.
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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.
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[The thesis of judicialization of health care by the elites: medication for mucopolysaccharidosis].
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.
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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
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Open field locomotor activity and anxiety-related behaviors in mucopolysaccharidosis type IIIA mice.
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.
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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.
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Directory of Open Access Journals (Sweden)
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
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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
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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.
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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).
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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
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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.
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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.
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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
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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.
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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
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Audiological findings in children with mucopolysaccharidoses type i-iv.
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.
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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
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Mucopolysaccharidosis I, II, and VI: Brief review and guidelines for treatment.
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.
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Mucopolysaccharidosis I, II, and VI: brief review and guidelines for treatment
Directory of Open Access Journals (Sweden)
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.
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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.
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Genetic and other diseases in the pottery of Tumaco-La Tolita culture in Colombia-Ecuador.
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.
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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
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Guanidinylated Neomycin Conjugation Enhances Intranasal Enzyme Replacement in the Brain.
Tong, Wenyong; Dwyer, Chrissa A; Thacker, Bryan E; Glass, Charles A; Brown, Jillian R; Hamill, Kristina; Moremen, Kelley W; Sarrazin, Stéphane; Gordts, Philip L S M; Dozier, Lara E; Patrick, Gentry N; Tor, Yitzhak; Esko, Jeffrey D
2017-12-06
Iduronidase (IDUA)-deficient mice accumulate glycosaminoglycans in cells and tissues and exhibit many of the same neuropathological symptoms of patients suffering from Mucopolysaccharidosis I. Intravenous enzyme-replacement therapy for Mucopolysaccharidosis I ameliorates glycosaminoglycan storage and many of the somatic aspects of the disease but fails to treat neurological symptoms due to poor transport across the blood-brain barrier. In this study, we examined the delivery of IDUA conjugated to guanidinoneomycin (GNeo), a molecular transporter. GNeo-IDUA and IDUA injected intravenously resulted in reduced hepatic glycosaminoglycan accumulation but had no effect in the brain due to fast clearance from the circulation. In contrast, intranasally administered GNeo-IDUA entered the brain rapidly. Repetitive intranasal treatment with GNeo-IDUA reduced glycosaminoglycan storage, lysosome size and number, and neurodegenerative astrogliosis in the olfactory bulb and primary somatosensory cortex, whereas IDUA was less effective. The enhanced efficacy of GNeo-IDUA was not the result of increased nose-to-brain delivery or enzyme stability, but rather due to more efficient uptake into neurons and astrocytes. GNeo conjugation also enhanced glycosaminoglycan clearance by intranasally delivered sulfamidase to the brain of sulfamidase-deficient mice, a model of Mucopolysaccharidosis IIIA. These findings suggest the general utility of the guanidinoglycoside-based delivery system for restoring missing lysosomal enzymes in the brain. Copyright © 2017 The American Society of Gene and Cell Therapy. Published by Elsevier Inc. All rights reserved.
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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
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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.
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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.
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Severity score system for progressive myelopathy: development and validation of a new clinical scale
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R.M. Castilhos
2012-07-01
Full Text Available Progressive myelopathies can be secondary to inborn errors of metabolism (IEM such as mucopolysaccharidosis, mucolipidosis, and adrenomyeloneuropathy. The available scale, Japanese Orthopaedic Association (JOA score, was validated only for degenerative vertebral diseases. Our objective is to propose and validate a new scale addressing progressive myelopathies and to present validating data for JOA in these diseases. A new scale, Severity Score System for Progressive Myelopathy (SSPROM, covering motor disability, sphincter dysfunction, spasticity, and sensory losses. Inter- and intra-rater reliabilities were measured. External validation was tested by applying JOA, the Expanded Disability Status Scale (EDSS, the Barthel index, and the Osame Motor Disability Score. Thirty-eight patients, 17 with adrenomyeloneuropathy, 3 with mucopolysaccharidosis I, 3 with mucopolysaccharidosis IV, 2 with mucopolysaccharidosis VI, 2 with mucolipidosis, and 11 with human T-cell lymphotropic virus type-1 (HTLV-1-associated myelopathy participated in the study. The mean ± SD SSPROM and JOA scores were 74.6 ± 11.4 and 12.4 ± 2.3, respectively. Construct validity for SSPROM (JOA: r = 0.84, P < 0.0001; EDSS: r = -0.83, P < 0.0001; Barthel: r = 0.56, P < 0.002; Osame: r = -0.94, P < 0.0001 and reliability (intra-rater: r = 0.83, P < 0.0001; inter-rater: r = 0.94, P < 0.0001 were demonstrated. The metric properties of JOA were similar to those found in SSPROM. Several clinimetric requirements were met for both SSPROM and JOA scales. Since SSPROM has a wider range, it should be useful for follow-up studies on IEM myelopathies.
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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
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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.
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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.
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Mean Platelet Volume as an Indicator of Platelet Rejuvenation Following Bone Marrow Transplantation.
1986-07-01
diameter N I Estes, 1968 Mucopolysaccharidosis diameter N I Estes, 1968 Osteogenesis imperfecta diameter N N Estes, 1968 Montreal Platelet Syndrome...6. Inherited Disorders of Connective Tissue: Platelet size was evaluated in 31 families with the following disorders: Osteogenesis imperfecta ...controlled by factors regulating the passage of platelets in and out of the pool. The splenic pool is known to be mobilized following exercise or epinephrine
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Assessment of dysmyelination with RAFFn MRI: application to murine MPS I.
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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.
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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
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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.
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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.
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Pericles Calias
Full Text Available A major challenge for the treatment of many central nervous system (CNS disorders is the lack of convenient and effective methods for delivering biological agents to the brain. Mucopolysaccharidosis II (Hunter syndrome is a rare inherited lysosomal storage disorder resulting from a deficiency of iduronate-2-sulfatase (I2S. I2S is a large, highly glycosylated enzyme. Intravenous administration is not likely to be an effective therapy for disease-related neurological outcomes that require enzyme access to the brain cells, in particular neurons and oligodendrocytes. We demonstrate that intracerebroventricular and lumbar intrathecal administration of recombinant I2S in dogs and nonhuman primates resulted in widespread enzyme distribution in the brain parenchyma, including remarkable deposition in the lysosomes of both neurons and oligodendrocytes. Lumbar intrathecal administration also resulted in enzyme delivery to the spinal cord, whereas little enzyme was detected there after intraventricular administration. Mucopolysaccharidosis II model is available in mice. Lumbar administration of recombinant I2S to enzyme deficient animals reduced the storage of glycosaminoglycans in both superficial and deep brain tissues, with concurrent morphological improvements. The observed patterns of enzyme transport from cerebrospinal fluid to the CNS tissues and the resultant biological activity (a warrant further investigation of intrathecal delivery of I2S via lumbar catheter as an experimental treatment for the neurological symptoms of Hunter syndrome and (b may have broader implications for CNS treatment with biopharmaceuticals.
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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
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Lysosomal Storage Diseases To date
HOFFMANN, Björn; MAYATEPEK, Ertan
2011-01-01
New therapeutic options and progress of approved therapies have made Lysosomal Storage Diseases (LSDs) one of the most exciting group of diseases. This review aims to summarize current achievements in these particular disorders and to give an outlook towards possible future treatment options. Enzyme replacement therapy is the gold standard for Gaucher disease, Fabry disease, Mucopolysaccharidosis type I, II, and VI, and for Pompe disease. Besides this, substrate reduction has been approved fo...
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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
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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.
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Baz, Ali Bulent; Akalin, Serdar; Arik, Hasan; Ergun, Ali
2012-01-01
Mucopolysaccharidosis IVA (MPS IVA: Morquio A syndrome) is a lysosomal storage disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Patients with MPS IVA appear healthy at birth. Morquio-specific radiographic changes can be observed prior to clinical signs and symptoms. Patients are usually affected by a severe joint degeneration from the 2nd or 3rd decade. Hyperlaxity of the joints is prominent due to the excess of intermediate metabolites. We report a patient with i...
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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.
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Directory of Open Access Journals (Sweden)
Susan Elliott
2016-09-01
Full Text Available In this data article we provide a detailed standard operating procedure for performing a tandem mass spectrometry, multiplex assay of 6 lysosomal enzymes for newborn screening of the lysosomal storage diseases Mucopolysaccharidosis-I, Pompe, Fabry, Niemann-Pick-A/B, Gaucher, and Krabbe, (Elliott, et al., 2016 [1]. We also provide the mass spectrometry peak areas for the product and internal standard ions typically observed with a dried blood spot punch from a random newborn, and we provide the daily variation of the daily mean activities for all 6 enzymes.
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International Nuclear Information System (INIS)
Petruschka, L.; Machill, G.; Wehnert, M.
1985-01-01
The investigation of 35 S-sulfate accumulation and of 35 SO 4 -labelled glycosaminoglucane molecules (chase-experiments) in cultured cells was used in post- and prenatal diagnosis of mucopolysaccharidosis (MPS). Cell lines which accumulate 35 S-sulfate can be differentiated by means of cross correction or measurement of enzyme activity. 34 patients with the MPS type I, II, III A, III B and VI, respectively, were diagnosed in this way. Four pregnancies at risk for MPS were prenatally monitored by the sulfate accumulation in cultured amniotic fluid cells. One fetus suffering from MPS II was found. (author)
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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
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2018-03-15
Adrenoleukodystrophy; Batten Disease; Mucopolysaccharidosis II; Leukodystrophy, Globoid Cell; Leukodystrophy, Metachromatic; Neimann Pick Disease; Pelizaeus-Merzbacher Disease; Sandhoff Disease; Tay-Sachs Disease; Brain Diseases, Metabolic, Inborn; Alpha-Mannosidosis; Sanfilippo Mucopolysaccharidoses
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Assessment of respiratory involvement in children with ...
African Journals Online (AJOL)
Mona M. El Falaki
2013-10-20
Oct 20, 2013 ... with mucoplysaccharidosis using pulmonary function tests ... was confirmed by unidimensional electrophoresis for GAGs in urine and/or specific enzymatic ... The mucopolysaccharidosis (MPS) are lysosomal storage dis-.
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Mucopolysaccharidoses Causing Valvular Heart Disease: Report and Review of Surgical Management.
Encarnacion, Carlos O; Hang, Dustin; Earing, Michael; Mitchell, Michael E
2017-01-01
Mucopolysaccharidosis type I is a genetic disorder with impaired glycosaminoglycan degradation. Cardiac pathologic involvement in this subset of patients is predominantly valvular heart disease. Valvular heart disease seen in these patients will most likely require surgical intervention in their lifetime. Only a limited amount of reports are dedicated to the cardiac surgical management of mucopolysaccharidoses. We present the case of a 32-year-old female with Hurler-Scheie syndrome who required multiple valve replacements due to progression of valvular dysfunction and decline in the quality of life. Multidisciplinary evaluation and discussion early are crucial for quality of life optimization in this cohort of patients.
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Bone marrow transplantation in patients with storage diseases: a developing country experience
Directory of Open Access Journals (Sweden)
Lange Marcos C.
2006-01-01
Full Text Available Bone marrow transplantation (BMT is a therapeutic option for patients with genetic storage diseases. Between 1979 and 2002, eight patients, four females and four males (1 to 13 years old were submitted to this procedure in our center. Six patients had mucopolysaccharidosis (MPS I in 3; MPS III in one and MPS VI in 2, one had adrenoleukodystrophy (ALD and one had Gaucher disease. Five patients had related and three unrelated BMT donor. Three patients developed graft versus host disease (two MPS I and one MPS VI and died between 37 and 151 days after transplantation. Five patients survived 4 to 16 years after transplantation. Three patients improved (one MPS I; one MPS VI and the Gaucher disease patient, one patient had no disease progression (ALD and in one patient this procedure did not change the natural course of the disease (MPS III.
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Assessment of respiratory involvement in children with ...
African Journals Online (AJOL)
Background: Mucopolysaccharidosis (MPS) are classified into seven clinical types based on eleven known lysosomal enzyme deficiencies of glycosaminoglycan (GAG) metabolism. Respiratory involvement seen in most MPS types includes recurrent respiratory infections, upper and lower airway obstruction, tracheomalacia ...
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... JavaScript. Mucopolysaccharidosis type III (MPS III) is a rare disease in which the body is missing or does not have enough of certain enzymes needed to break down long chains of sugar molecules. These chains of molecules are called glycosaminoglycans ( ...
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International Nuclear Information System (INIS)
Macy, D.W.; Gillette, E.L.; Gasper, P.W.; Thrall, M.A.; Wenger, D.A.; Kessell, M.L.; Hoover, E.A.
1984-01-01
Mucopolysaccharidosis VI is an inherited, metabolic defect in which a deficiency of arylsulfatase B, results in accumulation of glycosaminoglycans (GAG) in lysosomes. Arylsulfatase B activity was installed in an affected 2 year old siamese cat with no arylsulfatase B activity, excess urinary GAG, Alder-Reilly bodies in neutrophils, facial dysmorphia, corneal clouding, multiple epiphyseal dysplasia, and hind limb paresis. Following grafting of bone marrow from an immunologically nonreactive, female sibling with normal arylsulfatase B activity, increased arylsulfatase B activity and urinary excretion of hexuronic acid decreased by 19 days post transplantation. There were no metachromatic inclusions in circulating neutrophils, which were phenotypically female. The cat now has competent trilineage hematopoiesis, resolution of the facial dysmorphia, no corneal clouding, and improved movement of the head, neck, and mandible. The technique, sequence of hematologic recovery, and evidence of engraftment, are discussed. This may be a model for correction of mucopolysaccharidosis VI in man
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78 FR 63224 - Endocrinologic and Metabolic Drugs Advisory Committee; Notice of Meeting
2013-10-23
... treatment of Mucopolysaccharidosis Type IVA (Morquio A syndrome). Morquio A syndrome is a rare congenital... Agency's Web site at http://www.fda.gov/AdvisoryCommittees/default.htm and scroll down to the appropriate...Committees/Calendar/default.htm . Scroll down to the appropriate advisory committee meeting link. Procedure...
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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 ...
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African Journals Online (AJOL)
GB
This could have contributed to the refusal for readmission. In conclusion, identification and management of mucopolysaccharidosis type II in affected patients pose a problem in resource-constrained countries due to late identification and presentation, lack of facilities for diagnosis and treatment, as well as the cost and the.
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2002-01-01
BioMarin Pharmaceutical is developing laronidase, recombinant alpha-L-iduronidase enzyme replacement therapy for the treatment of mucopolysaccharidosis I (MPS-I) [the most severe form of this is called Hurler syndrome]. The company has received US and European orphan drug designation for the enzyme and has fast-track review status with the FDA. In 1998, BioMarin Pharmaceutical and Genzyme General formed a joint venture for development and marketing of laronidase. A Phase I trial in 10 patients with a range of disease severity of MPS-I required for US and European filing was completed at the Harbor-UCLA Medical Center in California. This open label trial involved weekly infusions with laronidase. The two-year follow-up data revealed sustained and, in certain parameters, improved clinical results recorded at the end of 1 year of therapy. BioMarin and Genzyme General have completed a pivotal, Phase III trial in the centres in the USA, Canada and Europe, including patients with Hurler-Scheie and Scheie syndromes. In a multicentre, double-blind, placebo-controlled study, all 45 patients with MPS-I have received at least their initial weekly infusion of laronidase. Patients are being evaluated over a 6-month period. BioMarin Pharmaceutical and Genzyme General have filed on 15 April 2002 the first portion of a 'rolling' BLA with the US FDA for use of laronidase in the treatment of MPS-I. The companies are planning to complete the BLA filing in Q3 2002. The application will include 6-month data from the ongoing open-label Phase III extension study and also the 6-month data from the placebo-controlled part of the Phase III study. In the open-label extension study, patients from both the treatment and placebo arms of the Phase III trial received weekly infusions of laronidase for at least 6 months. The response from the US FDA is anticipated during the H1 of 2003. Both companies plan to initiate two new clinical trials in patients with MPS-I. One study will enrol patients
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Genetics Home Reference: mucopolysaccharidosis type III
... 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 ...
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Genetics Home Reference: mucopolysaccharidosis type II
... 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 ...
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Genetics Home Reference: mucopolysaccharidosis type VI
... 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 ...
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Genetics Home Reference: mucopolysaccharidosis type IV
... 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, ...
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Genetics Home Reference: mucopolysaccharidosis type VII
... 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 ...
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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.
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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
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Directory of Open Access Journals (Sweden)
Heydy Bravo
2017-09-01
Full Text Available Lysosomal storage diseases (LSDs are genetic disorders, clinically heterogeneous, mainly caused by defects in genes encoding lysosomal enzymes that degrade macromolecules. Several LSDs already have specific therapies that may improve clinical outcomes, especially if introduced early in life. With this aim, screening methods have been established and newborn screening (NBS for some LSDs has been developed. Such programs should include additional procedures for the confirmation (or not of the cases that had an abnormal result in the initial screening. We present here the methods and results of the additional investigation performed in four babies with positive initial screening results in a program of NBS for LSDs performed by a private laboratory in over 10,000 newborns in Brazil. The suspicion in these cases was of Mucopolysaccharidosis I - MPS I (in two babies, Pompe disease and Gaucher disease (one baby each. One case of pseudodeficiency for MPS I, 1 carrier for MPS I, 1 case of pseudodeficiency for Pompe disease and 1 carrier for Gaucher disease were identified. This report illustrates the challenges that may be encountered by NBS programs for LSDs, and the need of a comprehensive protocol for the rapid and precise investigation of the babies who have an abnormal screening result.
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<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 -
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
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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>. -
The Burden Endured by Caregivers of Patients With Morquio A Syndrome
Christian J. Hendriksz MD; Christine Lavery; Mahmut Coker MD; Sema Kalkan Ucar MD; Mohit Jain PhD; Lisa Bell PhD; Christina Lampe MD
2014-01-01
This international survey performed by direct personal interview or mail evaluated the global burden among primary caregivers of patients with Morquio A syndrome. Collected outcomes included self-reported time spent on caregiving, proportion of daily activities (from the Mucopolysaccharidosis Health Assessment Questionnaire) requiring caregiver assistance, and how the patient’s age and wheelchair use affect these. In addition, the impact of caregiving on the caregivers’ relationship with fami...
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Navarrete-Martínez, Juana Inés; Limón-Rojas, Ana Elena; Gaytán-García, Maria de Jesús; Reyna-Figueroa, Jesús; Wakida-Kusunoki, Guillermo; Delgado-Calvillo, Ma Del Rocío; Cantú-Reyna, Consuelo; Cruz-Camino, Héctor; Cervantes-Barragán, David Eduardo
2017-05-01
To evaluate the results of a lysosomal newborn screening (NBS) program in a cohort of 20,018 Mexican patients over the course of 3years in a closed Mexican Health System (Petróleos Mexicanos [PEMEX] Health Services). Using dried blood spots (DBS), we performed a multiplex tandem mass spectrometry enzymatic assay for six lysosomal storage disorders (LSDs) including Pompe disease, Fabry disease, Gaucher disease, mucopolysaccharidosis type I (MPS-I), Niemann-Pick type A/B, and Krabbe disease. Screen-positive cases were confirmed using leukocyte enzymatic activity and DNA molecular analysis. From July 2012 to April 2016, 20,018 patients were screened; 20 patients were confirmed to have an LSD phenotype (99.9 in 100,000 newborns). Final distributions include 11 Pompe disease, five Fabry disease, two MPS-I, and two Niemann-Pick type A/B patients. We did not find any Gaucher or Krabbe patients. A final frequency of 1 in 1001 LSD newborn phenotypes was established. NBS is a major public health achievement that has decreased the morbidity and mortality of inborn errors of metabolism. The introduction of NBS for LSD presents new challenges. This is the first multiplex Latin-American study of six LSDs detected through NBS. Copyright © 2017 Elsevier Inc. All rights reserved.
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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 ...
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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 ...
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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 ...
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Wolfenden, C.; Wittkowski, A.; Hare, Dougal
2017-01-01
The prevalence of autism spectrum disorder (ASD) in many genetic disorders is well documented but not as yet in Mucopolysaccharidosis type III (MPS III). MPS III is a recessively inherited metabolic disorder and evidence suggests that symptoms of ASD present in MPS III. This systematic review examined the extant literature on the symptoms of ASD in MPS III and quality assessed a total of 16 studies. Results indicated that difficulties within speech, language and communication consistent with ...
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Home infusion with Elosulfase alpha (VimizimR) in a UK Paediatric setting
Niamh Finnigan; Jane Roberts; Jean Mercer; Simon A. Jones
2018-01-01
Enzyme replacement therapy is the only available treatment for Mucopolysaccharidosis type IVA (MPS IVA, Morquio syndrome). The treatment is lengthy and invasive involving weekly intravenous infusions of 4–5 h. This can cause significant disruption to normal family life so the provision of a safe and effective homecare service is essential. In order to deliver a safe service, robust standards must be in place; this includes appropriately trained members of homecare staff, detailed management f...
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<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 -
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
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Directory of Open Access Journals (Sweden)
Hilpold, A.
2009-12-01
Full Text Available <i>Centaurea subtilisi> from south east Italy and <i>C. exaratai> from south west Iberia were classified in the <i>Acrolophus- Phalolepisi> group and therein in section <i>Maculosae>. A molecular survey based on ITS sequence data indicates that both species should rather be placed in the <i>Jacea-Lepteranthus> group instead. This placement is consistent with the chromosome number of the two species, which is x = 11 like the rest of species of the <i>Jacea-Lepteranthus> group, and differs from the x = 9 of the other taxa included in sect. Maculosae. These results confi rm previous suggestions on the unnaturality of sect. <i>Maculosae. Centaurea exaratai> and <i>C. subtilisi> are quite different from the other species of <i>Jacea-Lepteranthus> in some striking morphological characters, which we hypothesize to be the result of parallel adaptation to dryer climates. The lack of competitors for pollination might be a good explanation for the partial or even total loss of showy flowers in these two species.
[es] <i>Centaurea subtilisi> del sureste de Italia y <i>C. exaratai> del suroeste de la Península Ibérica fueron clasificadas anteriormente en el grupo <i>Acrolophus-Phalolepis> y dentro de él en la sect. <i>Maculosae>. Una revisión molecular basada en secuencias de la región ITS indica que ambas deberían clasificarse en el grupo <i>Jacea-Lepteranthus>. Este cambio es coherente con el número cromosómico de las dos especies, que tienen x = 11 como el resto de las especies del grupo <i>Jacea-Lepteranthus> y no x = 9 como las especies del grupo <i>Acrolophus-Phalolepis>. Estos resultados confirman advertencias anteriores sobre el carácter artificial de la sect. <i>Maculosae. Centaurea exaratai> y <i>C. subtilisi> son bastante diferentes de las otras especies de <i>Jacea-Lepteranthus> en algunos caracteres morfológicos importantes, resultado, según nuestra hipótesis, de adaptaciones a -
Directory of Open Access Journals (Sweden)
Jonas Alex Morales Saute
Full Text Available ABSTRACT Hematopoietic stem cell transplantation (HSCT is the only available treatment for the neurological involvement of disorders such as late-onset metachromatic leukodystrophy (MLD, mucopolysaccharidosis type I-Hurler (MPS-IH, and X-linked cerebral adrenoleukodystrophy (CALD. Objective To describe survival and neurological outcomes after HSCT for these disorders. Methods Seven CALD, 2 MLD and 2 MPS-IH patients underwent HSCT between 2007 and 2014. Neurological examinations, magnetic resonance imaging, molecular and biochemical studies were obtained at baseline and repeated when appropriated. Results Favorable outcomes were obtained with 4/5 related and 3/6 unrelated donors. Two patients died from procedure-related complications. Nine transplanted patients were alive after a median of 3.7 years: neurological stabilization was obtained in 5/6 CALD, 1/2 MLD, and one MPS-IH patient. Brain lesions of the MPS-IH patient were reduced four years after HSCT. Conclusion Good outcomes were obtained when HSCT was performed before adulthood, early in the clinical course, and/or from a related donor.
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β-Galactosidase Deficiency in Colombia
Directory of Open Access Journals (Sweden)
Alfredo Uribe PhD
2015-05-01
Full Text Available β-Galactosidase (BGal is the first enzyme involved in the catabolism of sphingolipids. Two pathologies have been directly associated with its deficiency: GM1 gangliosidosis and Morquio B. Morquio B is among the rarest types of mucopolysaccharidosis (MPS. We aim to document the β-galactosidase deficiency in Colombia. We evaluated leukocytes from 1492 healthy Colombian individuals and 923 patients, referred between 2005 and August 2014. Dried blood spot (DBS samples from the same number of patients were evaluated. β-Galactosidase was measured with 4-methylumbelliferyl-β- d -galactoside. As a control enzyme, the total hexosaminidase activity was also evaluated. We identified 14 patients with GM1 gangliosidosis, 5 patients with Morquio B, and 1 patient with I-cell disease. We could establish a reference value for Bgal in Colombian leukocyte samples. GM1 gangliosidosis is the main pathology associated with a direct deficiency of BGal. The high number of patients found with MPS IVB indicates that there are patients who could be misdiagnosed due to an unawareness of the disease.
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<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>. -
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
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Successful airway management with King Vision device in a child with Morquio syndrome: case report
Directory of Open Access Journals (Sweden)
Lina Maritza Guerra
2017-09-01
Full Text Available Morquio syndrome also called type IV mucopolysaccharidosis, is a condition produced by lysosomal deposit. Morquio syndrome have several implications in the airway management because is characterized by C1-C2, instability, short height, cervical spine instability, odontoid hypoplasia, and Pectus carinatum, this, in addition to airway anatomy distortion. Case summary: This is a case report of successful airway management with video laryngoscopy of a child whit anticipated difficult airway whit Morquio syndrome. Conclusion: The video laryngoscopes are a good choice for management of anticipated difficult airway in child patients.
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Directory of Open Access Journals (Sweden)
M. F.S. RAMOS
2009-01-01
Full Text Available
A utilização de extratos vegetais em produtos farmacêuticos e cosméticos tem mostrado ser uma tendência mundial e cresceu substancialmente nas duas últimas décadas. No entanto, há ainda poucos relatos na literatura com relação à atividade mutagênica ou fototóxica de extratos vegetais. No presente trabalho foi avaliada a atividade fototóxica e o “screening” mutagênico de extratos fluidos e secos de própolis, <i>Aloe> spp. e <i>Hamamelis virginianai>. Na investigação de fototoxicidade foram realizados ensaios microbiológicos, utilizando cepas de <i>Candida albicansi> e <i>Saccharomyces cerevisiaei>, bem como ensaios biológicos com cobaias albinos. Extratos etanólicos de <i>Ruta graveolensi> e <i>Citrus> spp., além de 8-metoxipsoraleno (fármaco sintético padrão, foram usados como controles positivos em ambos os testes. A atividade mutagênica foi avaliada qualitativamente segundo o “spot test” descrito por Maron & Ames, com cepas de <i>Salmonella typhimuriumi> TA97, TA98, TA100 e TA102, empregando como controle positivo o óxido de 4-nitroquinolina. Não foi observada atividade fototóxica, em ambos os ensaios realizados, para qualquer dos extratos. O ensaio microbiológico demonstrou uma atividade fungistática ou fungicida nos extratos secos de hamamélis. Os resultados obtidos nos ensaios microbiológicos com a levedura <i>S. cerevisiaei> indicam que este microrganismo apresentou eficiência no procedimento de “screening” de atividade fototóxica comparável à obtida com <i>C. albicansi>. Os extratos vegetais não apresentaram atividade mutagênica nos ensaios preliminares realizados. Palavras-chave: <i>Aloe> spp., <i>Hamamelis virginianai>, própolis, fototoxicidade, mutagenicidade.
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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. -
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...
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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.
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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. -
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...
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Hoog, Andrew
2011-01-01
As sales and usage of iPhones increase so does the demand on organizations that conduct examinations on this device. iPhone and iOS Forensics takes an in-depth look at methods and processes that analyze the iPhone/iPod in an official legal manner. All of the methods and procedures outlined in the book can be taken into any court room. This book details the iPhone with information data sets that are new and evolving, with official hardware knowledge from Apple itself to help aid investigators. iPhone market share has increased to 50% of worldwide mobile phone usageEmployment in digital fo
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Directory of Open Access Journals (Sweden)
Aragón Sandra
2007-06-01
Full Text Available
<i>Praon> pos. <i>occidentale> es un parasitoide promisorio para regular poblaciones de áfidos <i>Macrosiphum euphorbiaei> en cultivos comerciales bajo invernadero de rosas. Se evaluó la capacidad parasítica de <i>P.> pos. <i>occidentale> bajo tres temperaturas constantes (18, 25 y 28ºC y variando la densidad de su hospedero (5, 10, 20, 40, 80 y 150. En cada unidad experimental se liberó una pareja del parasitoide con 24 horas de edad y se mantuvieron durante 24 horas en incubadoras graduadas a cada temperatura en evauación, con 12 horas de luz y 12 de oscuridad. Se registró el número de áfidos parasitados y se permitió el desarrollo de los huevos depositados hasta adulto. Se estimó la tasa instantánea de búsqueda (a’ y el tiempo de manipulación (Th a partir de los cuales se ajustó el modelo de respuesta funcional tipo II para cada temperatura. La tasa instantánea de búsqueda (a’ fue más alta a 18ºC con un valor de 0,1081, seguida de 28ºC con 0,0323 y 25ºC con 0,0103. El tiempo de manipulación (Th más corto fue el que se presentó a 25ºC de 4,8913, seguido de 28º C con un tiempo de 5,7579 y 18ºC con 8,2697. El máximo número de individuos parasitados estimado fue de 4,9 a 25ºC. A 18ºC el 60% de los áfidos parasitados alcanzó la emergencia del adulto, el 74,2% a 25ºC y el 88% a 28ºC. No existe ningún efecto significativo de la densidad del hospedero ni de la temperatura en la proporción sexual de <i>Praon> pos. <i>Occidentale>.
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Directory of Open Access Journals (Sweden)
Oľga Danišová
2015-05-01
Full Text Available Increased risk of zoonotic transmission of the potential human pathogenic species [i]Enterocytozoon bieneusi[/i], [i]Encephalitozoon intestinalis[/i] and [i]Encephalitozoon cuniculi [/i]was detected in wild immunocompetent mice (Mus musculus musculus; n=280. Analysis was conducted with the use of PMP1/PMP2 primers and SYBR Green RT-PCR. Using Real Time PCR and comparing the sequences with sequences in the GenBank, [i]E. bieneusi[/i] was detected in 3 samples (1.07 %, [i]E. cuniculi [/i]in 1 sample (0.35 % and [i]E. intestinalis[/i] in 1 sample (0.35 %. The results of this report document the low host specificity of detected microsporidia species, and imply the importance of synanthropic rodents as a potential source of human microsporidial infection.
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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 ...
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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.
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Programming iOS 4 Fundamentals of iPhone, iPad, and iPod touch Development
Neuburg, Matt
2011-01-01
Get a solid grounding in all the fundamentals of Cocoa Touch, and avoid problems during iPhone and iPad app development. With Programming iOS 4, you'll dig into Cocoa and learn how to work effectively with Objective-C and Xcode. This book covers iOS 4 in a rigorous, orderly fashion-ideal whether you're approaching iOS for the first time or need a reference to bolster existing skills. Learn Objective-C language details and object-oriented programming conceptsUnderstand the anatomy of an Xcode project and all the stages of its lifecycleGrasp key Cocoa concepts such as relationships between clas
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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 -
Composition and thermochemistry of the equilibrium vapour of the systems NaI-FeI2 and NaI-PbI2
International Nuclear Information System (INIS)
Hilpert, K.; Gerads, H.; Koberts, D.; Miller, M.
1987-01-01
The vaporization of NaI/FeI 2 and NaI/PbI 2 samples of equimolar composition was investigated was investigated in the temperature ranges between 574 to 683 K and 562 to 669 K, respectively, by using the mass spectrometric Knudsen effusion method. The gaseous species I, I 2 , NaI, (NaI) 2 , FeI 2 , (FeI 2 ) 2 , FeI 3 , NaFeI 3 , and Na 2 FeI 4 (NaI-FeI 2 system) as well as NaI, (NaI) 2 , PbI 2 , (PbI 2 ) 2 , and NaPbI 3 (NaI-PbI 2 system) are present in the equilibrium vapours. The equilibrium partial pressures of these species were determined with the exception of I, I 2 , and FeI 3 . Enthalpies and entropies of dissociation resulted for the reactions NaFeI 3 (g) ↔ NaI(g)+FeI 2 (g) (1), Na 2 FeI 4 (g) ↔ 2NaI(g)+FeI 2 (g) and (2) NaPbI 3 (g) ↔ NaI(g)+PbI 2 (g) (3) as Δ d H 298 0 (Eq. (1)) = 184±5 kJ mol -1 , Δ d S 298 0 (Eq. (1)) = 143±8 J mol -1 K -1 ; Δ d H 298 0 (Eq. (2)) = 333±9 kJ mol -1 , Δ d S 298 0 (Eq. (2)) = 274±14 J mol -1 K -1 ; and Δ d H 298 0 (Eq. (3)) = 168±5 kJ mol -1 , Δ d S 298 0 (Eq. (3)) = 151±9 J mol -1 K -1 . Equilibrium constants for these reactions are additionally given. The pressures of NaFeI 3 (g) and NaPbI 3 (g) as all as their enthalpies of dissociation are discussed with respect to their significance of semi empirical rules. (orig.)
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Kina i Zambia: Indsigt i kinesiske investeringer i Zambias kobberminer
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,...
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Directory of Open Access Journals (Sweden)
Schulte Christopher
2011-12-01
Full Text Available Hoog, A., & Strzempka, K. (2011. iPhone and iOS Forensics: Investigation, Analysis and Mobile Security for Apple iPhone, iPad and iOS Devices. Waltham, MA: Syngress. 336 pages, ISBN: 978-1-59749-659-9, US$69.95.Reviewed by Christopher Schulte, EnCE & ACE, LuciData Inc., Minneapolis, Minnesota (cschulte@lucidatainc.comThese are exciting times for Digital Forensics practitioners. While our examinations of mobile devices (including cell phones and tablet computers continue to bring new and sometimes hair-pulling challenges into our labs and on-site engagements, research and understanding of these tiny computers is increasing at what seems an exponential rate. This is especially true in the iOS (Apple Computer’s mobile operating system that powers the iPhone, iPad, iPod Touch and Apple TV space. The diligent work of talented computer scientists in this field allows examiners everywhere to reap the benefits of easier, faster and more effective examinations that yield more accurate and defendable results.(see PDF for full review
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NITRATI I NITRITI, METABOLIZAM I TOKSIČNOST
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...
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Miller, Patricia A; Mulla, Sohail M; Adams-Webber, Thomasin; Sivji, Yasmin; Guyatt, Gordon H; Johnston, Bradley C
2016-01-01
To investigate the use, challenges and opportunities associated with using patient-reported outcomes (PROs) in studies with patients with rare lysosomal storage diseases (LSDs), we conducted interviews with researchers and health technology assessment (HTA) experts, and developed the methods for a systematic review of the literature. The purpose of the review is to identify the psychometrically sound generic and disease-specific PROs used in studies with patients with five LSDs of interest: Fabry, Gaucher (Type I), Niemann-Pick (Type B) and Pompe diseases, and mucopolysaccharidosis (Types I and II). Researchers and HTA experts who responded to an email invitation participated in a telephone interview. We used qualitative content analysis to analyze the anonymized transcripts. We conducted a comprehensive literature search for studies that used PROs to investigate burden of disease or to assess the impact of interventions across the five LSDs of interest. Interviews with seven researchers and six HTA experts representing eight countries revealed five themes. These were: (i) the importance of using psychometrically sound PROs in studies with rare diseases, (ii) the paucity of disease-specific PROs, (iii) the importance of having PRO data for economic analyses, (iv) practical and psychometric limitations of existing PROs, and (v) suggestions for new PROs. The systematic review has been completed. The interviews highlight current challenges and opportunities experienced by researchers and HTA experts involved in work with rare LSDs. The ongoing systematic review will highlight the experience, opportunities, and limitations of PROs in LSDs and provide suggestions for future research.
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iOS 4 Programming Cookbook Solutions & Examples for iPhone, iPad, and iPod touch Apps
Nahavandipoor, Vandad
2011-01-01
You can build a variety of amazing apps on the iOS platform-and every one of them presents a unique set of problems. With the recipes in this cookbook, you'll go beyond theory to solve the vexing, real-life issues you're likely to face when creating apps for the iPhone, iPad, or iPod Touch. Each recipe provides a clear solution and sample code that you can use right away. You'll find solutions for working with development frameworks in iOS SDK 4 and technologies such as Cocoa, Objective-C, Xcode, and Interface Builder. Whether you have a little or a lot of experience with iOS development, yo
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Garfinkel, Simson
2013-01-01
Hoog, A., & Strzempka, K. (2011). iPhone and iOS Forensics: Investigation, Analysis and Mobile Security for Apple iPhone, iPad and iOS Devices. Waltham, MA: Syngress. 336 pages, ISBN: 978-1-59749-659-9, US$69.95.Reviewed by Christopher Schulte, EnCE & ACE, LuciData Inc., Minneapolis, Minnesota ()These are exciting times for Digital Forensics practitioners. While our examinations of mobile devices (including cell phones and tablet computers) continue to bring ne...
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Schulte Christopher
2011-01-01
Hoog, A., & Strzempka, K. (2011). iPhone and iOS Forensics: Investigation, Analysis and Mobile Security for Apple iPhone, iPad and iOS Devices. Waltham, MA: Syngress. 336 pages, ISBN: 978-1-59749-659-9, US$69.95.Reviewed by Christopher Schulte, EnCE & ACE, LuciData Inc., Minneapolis, Minnesota ()These are exciting times for Digital Forensics practitioners. While our examinations of mobile devices (including cell phones and tablet computers) continue to bring ne...
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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
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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...
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Directory of Open Access Journals (Sweden)
Molero, Julià
1992-12-01
Full Text Available We present a taxonomic study of the representatives or <i>Euphorbia> subsect. <i>Esula> in the Iberian Peninsula. Prior to this, a first section is included on the study of the leaf surface and a second section on chromosome numbers.
The section on leaf surface is based on a study of the leaves or 45 populations of Iberian and European taxa of the subsections using a light microscope and SEM. The characters analyzed are cell shape, morphology of the cells and stomata (primary and secondary sculpture and epicuticular waxes (tertiary sculpture. Some microcharacters of the leaf surface proved particularly usefu1for taxonomical purposes. Thus the basic type of stoma and the distribution model of the stomata on the two sides of the leaf are characters which make it possible to separate taxa as closely related as <i>E. esulai> L. subsp. <i>esula> and <i>E. esulai> L. subsp <i>orientalis> (Boiss. in DC. Molero & Rovira. The morphological type of the epicuticular waxes also enables us to differentiate between <i>E.graminifolia> Vill. and <i>E. esulai> aggr. And to distinguish subsp. <i>bolosii> Molero & Rovira from the remaining subespecies in <i>E. nevadensisi> Boiss. & Reuter.
Cytogenetic investigation reveals the presence of only the diploid cytotype (2n=10 in <i>E. cyparissiasi> L. and <i>E. esulai> L. subsp. <i>esula> in the Iberian Peninsula. We describe for the first time in <i>E. nevadensisi> s.1. a polyploidy complex with a base of x= 10 in which the diploid level (2n=20 is present in all subspecies; the tetraploid level (2n=40 is present in <i>E. nevadensisi> subsp. <i>nevadensis> and the hexaploid level (2n=60 is found in <i>E. nevadensisi> subsp. <i>bolosii>. Chromosome number is not a parameter that can be used for taxonomic purposes. In <i>E. nevadensisi>, cytogenetic differentiation has followed its own course, with no apparent relationship to the process of morphological -
Directory of Open Access Journals (Sweden)
Simson Garfinkel
2013-12-01
Full Text Available Hoog, A., and Strzempka, K. (2011. iPhone and iOS Forensic: Investigation, Analysis and Mobile Security for Apple iPhone, iPad and iOS Devices. Syngress, Elsevier, xv + 310 pages; ISBN-10: 1597496596; ISBN-13: 978-1597496599, $69.95Reviewed by Simson Garfinkel, Naval Postgraduate SchoolIn April 2011 news outlets around the world revealed shocking news about Apple’s iPhone: for reasons that were not apparently clear, every iPhone contained a small SQLite database that logged where and when the user had been whenever the phone was turned on, and those records went back for pretty much as long as the user had owned their phone. Apple eventually declared that the data cache was the result of a bug and issued a software update to prune the database (it had previously grown without limit. Privacy activists rejoiced that their beloved iPhones were once again trustworthy. But forensics examiners just shook their heads: many had known about the iPhone’s tracking capabilities for more than a year and had kept quiet. They had made good use of that data. Apple’s pro-privacy patch was actually a setback for law enforcement.(see PDF for full review
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Una nueva <i>Armeria> del Sistema Central (España: <i>A. rivasmartineziii> (Plumbaginaceae
Directory of Open Access Journals (Sweden)
Sardinero, Santiago
1997-12-01
Full Text Available A new taxon from the central-western Sistema Central (Sierra de Béjar and Sierra de Tormantos, Spain, <i>Armeria rivasmartineziii>. is described and illustrated. lts morphological affinities and variability are discussed in concordance with its hypothetical hybrid origin.
Se describe e ilustra un nuevo taxon del centro-occidente del Sistema Central español. <i>Armeria rivasmartineziii>. Se discuten sus afinidades morfológicas y variabilidad en concordancia con su hipotético origen híbrido. -
Book Review: iOS Forensic Analysis: For iPhone, iPad and iPod Touch
Directory of Open Access Journals (Sweden)
Christopher Schulte
2011-03-01
Full Text Available As Digital Forensics practitioners, we know that our discipline is constantly evolving. Keeping abreast means we need to continually refine and broaden our knowledge pools through experience, education, research, peer exchange, and more. Mobile device forensics can be especially dynamic and challenging. With multiple standards in place at the hardware, operating system, and user interface levels, it can be daunting to preserve, analyze, search and report on these tiny yet ubiquitous hand-held computers. Apple Computer’s line of mobile products (iOS devices - iPhone, iPad, iPod Touch is no exception to this rule.(see PDF for full review
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Copper(I), silver(I) and gold(I) halide complexes with the dithioformamidinium dihalides
Peyronel, Giorgio; Malavasi, Wanda; Pignedoli, Anna
Some copper(I), silver(I) and gold(I) halide complexes with the dithioformamidinium dihalides (Tu 2X 2) were prepared and studied by infrared spectroscopy and conductometry: 3CuX.2Tu 2X 2(XCl,I), CuBr.Tu 2Br 2, 4CuBr.3.5Tu 2Br 2.MeOH, 2CuBr.Tu 2Br 2.0.66EtOH, 3CuI.2Tu 2I 2, 2AgCl.2.5Tu 2Cl 2, 3AgCl.2Tu 2Cl 2.0.5EtOH, 3AgCl.Tu 2Cl 2, 2AgBr.2Tu 2Br 2.0.5Tu 2(NO 3) 2.H 2O, AgBr.Tu 2Br 2, 4AgBr.Tu 2Br 2, 4AgI.0.5Tu 2I 2.EtOH, AuCl.1.5Tu 2Cl 2, 4AuCl.3.5Tu 2Cl 2.2DMF, AuBr.4Tu 2Br 2, AuBr.2Tu 2Br 2.1.5DMF, AuI.5Tu 2I 2, AuI.Tu 2I 2. A decrease of the ν(NH), δ(NH 2) and ν(CN 2) frequencies and an increase of the ν(CS) frequencies indicate an N-coordination of the dithioformamidinium cation to the metal ions; ν(MN) and ν(MX) frequencies are tentatively assigned in the far-infrared spectra.
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Take control of mail on the iPad, iPhone, and iPod Touch
Kissell, Joe
2010-01-01
Questions answered in this ebook include: Why is an IMAP account especially useful on a mobile device? How do I set up my email accounts? How do I move around in the Mail app? How do I set up mailboxes for effective navigation and filing? How do I handle attachments? How does Mail integrate with other apps, like Calendar and Contacts? What are the best ways to find messages in the Mail app? What's the deal with Exchange/ActiveSync accounts? Should I push or fetch my messages? How do I integrate Gmail with the Mail app? Help! I can't send my email... what should I do?
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Vitamin D receptor gene Alw I, Fok I, Apa I, and Taq I polymorphisms in patients with urinary stone.
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.
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Inner ionization in A sup I sup I B sup V sup I
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
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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
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Supplementary Material for: Tukey <i>g-and-h> Random Fields
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.
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iMovie '09 & iDVD pocket genius
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.
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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...
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Hunter′s syndrome: A case report
Directory of Open Access Journals (Sweden)
N S Savitha
2015-01-01
Full Text Available Hunter′s syndrome or mucopolysaccharidosis (MPS type II is an X-linked recessive mucopolysaccharide disorder caused by a defect in the metabolism of glycosaminoglycans (GAGs characterized by involvement of nervous, cardiovascular, respiratory, and mucoskeletal systems along with numerous oral manifestations. This is a case report of a 13-year-old boy referred to the Department of Pediatric Dentistry with a chief complaint of irregularly placed teeth from a general physician. Here we highlight the pivotal role of pediatric dentists in diagnosis and treatment planning for patients diagnosed with such systemic conditions and the provision of advanced dental care in the management of the same.
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Saito, M; Takenouchi, Y; Kunisaki, N; Kimura, S
2001-05-01
The subunit compositions of skin and muscle type I collagens from rainbow trout were found to be alpha1(I)alpha2(I)alpha3(I) and [alpha1(I)](2)alpha2(I), respectively. The occurrence of alpha3(I) has been observed only for bonyfish. The skin collagen exhibited more susceptibility to both heat denaturation and MMP-13 digestion than the muscle counterpart; the former had a lower denaturation temperature by about 0.5 degrees C than the latter. The lower stability of skin collagen, however, is not due to the low levels of imino acids because the contents of Pro and Hyp were almost constant in both collagens. On the other hand, some cDNAs coding for the N-terminal and/or a part of triple-helical domains of proalpha(I) chains were cloned from the cDNA library of rainbow trout fibroblasts. These cDNAs together with the previously cloned collagen cDNAs gave information about the complete primary structure of type I procollagen. The main triple-helical domain of each proalpha(I) chain had 338 uninterrupted Gly-X-Y triplets consisting of 1014 amino acids and was unique in its high content of Gly-Gly doublets. In particular, the bonyfish-specific alpha(I) chain, proalpha3(I) was characterized by the small number of Gly-Pro-Pro triplets, 19, and the large number of Gly-Gly doublets, 38, in the triple-helical domain, compared to 23 and 22, respectively, for proalpha1(I). The small number of Gly-Pro-Pro and the large number of Gly-Gly in proalpha3(I) was assumed to partially loosen the triple-helical structure of skin collagen, leading to the lower stability of skin collagen mentioned above. Finally, phylogenetic analyses revealed that proalpha3(I) had diverged from proalpha1(I). This study is the first report of the complete primary structure of fish type I procollagen.
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Energy Technology Data Exchange (ETDEWEB)
Park, G.; Koo, B.-C. [Department of Physics and Astronomy, Seoul National University, 1 Gwanak-ro, Gwanak-gu, Seoul 151-742 (Korea, Republic of); Gibson, S. J.; Newton, J. H. [Department of Physics and Astronomy, Western Kentucky University, Bowling Green, KY 42101 (United States); Kang, J.-H.; Lane, D. C.; Douglas, K. A. [Arecibo Observatory, HC 3 Box 53995, Arecibo, PR 00612 (United States); Peek, J. E. G. [Department of Astronomy, Columbia University, New York, NY 10027 (United States); Korpela, E. J. [Space Sciences Laboratory, University of California, Berkeley, CA 94720 (United States); Heiles, C., E-mail: koo@astro.snu.ac.kr [Radio Astronomy Lab, UC Berkeley, 601 Campbell Hall, Berkeley, CA 94720 (United States)
2013-11-01
We search for fast-expanding H I shells associated with Galactic supernova remnants (SNRs) in the longitude range l ≈ 32° to 77° using 21 cm line data from the Inner-Galaxy Arecibo L-band Feed Array (I-GALFA) H I survey. Among the 39 known Galactic SNRs in this region, we find such H I shells in 4 SNRs: W44, G54.4-0.3, W51C, and CTB 80. All four were previously identified in low-resolution surveys, and three of those (excluding G54.4-0.3) were previously studied with the Arecibo telescope. A remarkable new result, however, is the detection of H I emission at both very high positive and negative velocities in W44 from the receding and approaching parts of the H I expanding shell, respectively. This is the first detection of both sides of an expanding shell associated with an SNR in H I 21 cm emission. The high-resolution I-GALFA survey data also reveal a prominent expanding H I shell with high circular symmetry associated with G54.4-0.3. We explore the physical characteristics of four SNRs and discuss what differentiates them from other SNRs in the survey area. We conclude that these four SNRs are likely the remnants of core-collapse supernovae interacting with a relatively dense (∼> 1 cm{sup –3}) ambient medium, and we discuss the visibility of SNRs in the H I 21 cm line.
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<i>Fucus> and <i>Ascophyllum> seaweeds are significant contributors to coastal iodine emissions
H. Berresheim; C. D. O'Dowd; F. C. Küpper; P. P. A. Smyth; J. Chen; A. L. Vogel; D. Ceburnis; D. S. Venables; M. Kundel; U. R. Thorenz; R.-J. Huang; T. Hoffmann
2012-01-01
Based on the results of a pilot study in 2007, which found high mixing ratios of molecular iodine (I2) above the intertidal macroalgae (seaweed) beds at Mweenish Bay (Ireland), we extended the study to nine different locations in the vicinity of Mace Head Atmospheric Research Station on the west coast of Ireland during a field campaign in 2009. I2 mixing ratios from 104 to 393 ppt were found above the macroalgae beds, implying a high source strength of I2. Such mixing ratios are suffic...
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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
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Pro Core Data for iOS Data Access and Persistence Engine for iPhone, iPad, and iPod Touch
Privat, M
2011-01-01
The power of Core Data allows iOS developers to efficiently store and retrieve application data using familiar object-oriented paradigms. Pro Core Data for iOS explains both how and why to use Core Data for data storage, from simple to advanced techniques. Covering common and advanced persistence patterns, this book prepares any iOS developer to store and retrieve data accurately and proficiently. Lots of iOS development books touch on Core Data, taking you through a few mainstream use cases for storing and retrieving data in your iOS applications. In Pro Core Data for iOS, however, we take yo
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Viis aastat iPhone’i / Tõnu Ojala
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
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Professional iPhone and iPad Database Application Programming
Alessi, Patrick
2010-01-01
A much-needed resource on database development and enterprise integration for the iPhone. An enormous demand exists for getting iPhone applications into the enterprise and this book guides you through all the necessary steps for integrating an iPhone app within an existing enterprise. Experienced iPhone developers will learn how to take advantage of the built-in capabilities of the iPhone to confidently implement a data-driven application for the iPhone.: Shows you how to integrate iPhone applications into enterprise class systems; Introduces development of data-driven applications on the iPho
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Antioksidacijska svojstva i stabilnost boje crvenog piva, ružičastog vina i ružičastog pjenušca
Šipalo, Toni
2016-01-01
Biološki aktivne supstancije u pivu i vinu, posebice fenoli, zbog antioksidacijske aktivnosti smanjuju rizik za oboljenje od sve učestalijih kroničnih bolesti. Udjel fenolnih spojeva jedan je od najvažnijih parametara sastava, kako piva, tako i vina, budući da doprinose njihovim organoleptičkim svojstvima, kao što su boja, trpkost i gorčina. U ovom su radu uspoređena antioksidacijska svojstva Istarskog crvenog piva, ružičastog vina i ružičastog pjenušca primjenom različitih testova: određivan...
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A designated centre for people with disabilities operated by Ability West, Galway
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
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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...
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Pickling process of capers (<i>Capparis spp.i> flower buds
Directory of Open Access Journals (Sweden)
Özcan, Musa
1999-04-01
Full Text Available Middle sized (8 < x < 13 mm buds of <i>Capparis spinosai> var. <i>spinosa> and C. <i>ovata> var. <i>canescens> from June in brines containing 5,10,15 and 20% salt and from August in brines of 15% salt, and three different size (x < 8 mm, 8 < x < 13 mm, x > 13 mm buds of C. . <i>ovata> var. <i>canescens> from June in brines of 15% salt were pickled for two months fermentation. Some chemical and microbiological analyses were done in brines during fermentation. Most suitable salt concentration for lactic acid bacteria (LAB activity were 5% and partly 10%. Acidity, LAB activity, sedimentation and hardness were reduced by increasing bud size in C. <i>ovata>. Small buds of C. <i>ovata> for pickling product had advantage for colour and flavour, however, more sediment and partly softening showed disadvantage. For both species, pickling time was determined as 40 to 50 days in regard of end-product flavour and odour, brine acidity and pH, and LAB activity.
Se encurtieron durante dos meses botones florales de tamaño medio (8 < x < 13 mm de <i>Capparis spinosai> var. <i>spinosa> y C. <i>ovata> var. <i>canescens>, los recolectados en Junio en salmueras conteniendo 5, 10, 15 y 20% de sal, y los de Agosto en salmueras de 15% de sal; y tres tamaños diferentes (x < 8 mm, 8 < x < 13 mm, X > 13 mm de C. <i>ovata> var. <i>canescens> de Junio en salmueras de 15% de sal. Se realizaron algunos análisis químicos y microbiológicos durante la fermentación. Las concentraciones de sal más adecuadas para la actividad de las bacterias del ácido láctico (LAB fueron 5% y parcialmente 10%. Acidez, actividad de LAB, sedimentación y firmeza (hardness se redujeron al incrementar el tamaño de las alcaparras de C. <i>ovata>. Los tamaños pequeños de C. <i>ovata> presentaron en el producto encurtido ventajas en color y sabor, pero desventajas por más sedimento y ablandamiento parcial. El tiempo de encurtido para ambas -
Investigation and thermodynamic calculation of phase diagram of CdI2-PbI2-NaI system
International Nuclear Information System (INIS)
Storonkin, A.V.; Vasil'kova, I.V.; Korobkov, S.V.
1976-01-01
Using the thermographic and X-ray phase analyses binary CdI 2 -PbI 2 , PI 2 -NaI, CdI 2 -NaI systems and a triple CdI 2 -PbI 2 -NaI system are investigated and their melting diagrams are plotted. A method of thermodynamic calculation has been proposed and tested of the shape of the eutectic lines for the system CdI 2 -PbI 2 -NaI, taking into account the non-ideality of the liquid phase. The method uses data obtained for the binary systems. The liquidus surface of the triple system has been constructed on the basis of the calculation. The results of the calculation of the triple eutectics are in good agreement with the experimental data
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The business of iOS app development for iPhone, iPad and iPod Touch
Wooldridge, Dave
2014-01-01
Updated and expanded for the new Apple iOS8, The Business of iOS App Development, Third Edition shows you how to incorporate marketing and business savvy into every aspect of the design and development process, giving your app the best possible chance of succeeding in the App Store. This book is written by experienced developers with business backgrounds, taking you step-by-step through cost-effective marketing techniques that have proven successful for professional iOS app creators-perfect for independent developers on shoestring budgets. No prior business knowledge is required. The pheno
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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.
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Energy Technology Data Exchange (ETDEWEB)
Chen, Dennis P.; Skrabalak, Sara E.
2016-04-18
(Ga1–<i>x>)(N1–<i>x> O <i>x>) (GZNO) particles with enhanced optical absorption were synthesized by topotactic transformation of Zn2+/Ga3+ layered double hydroxides. This outcome was achieved by suppressing Zn volatilization during nitridation by maintaining a low partial pressure of O2 (pO2). Zn-rich (x > 1/3) variants of GZNO were achieved and compared to those prepared by conventional ammonoylsis conditions. The optical absorption and structural properties of these samples were compared to those prepared in the absence of O2 by diffuse-reflectance spectroscopy and powder X-ray diffraction methods. Notably, suppression of Zn volatilization leads to smaller-band-gap materials (2.30 eV for x = 0.42 versus 2.71 eV for x = 0.21) and reduced structural defects. This synthetic route and set of characterizations provide useful structure–property studies of GZNO and potentially other oxynitrides of interest as photocatalysts.
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Skew scattering dominated anomalous Hall effect in Co<i>x>(MgO)100-<i>x> granular thin films
Zhang, Qiang; Wen, Yan; Zhao, Yuelei; Li, Peng; He, Xin; Zhang, Junli; He, Yao; Peng, Yong; Yu, Ronghai; Zhang, Xixiang
2017-01-01
We investigated the mechanism(s) of the anomalous Hall effect (AHE) in magnetic granular materials by fabricating 100-nm-thick thin films of Co<i>x>(MgO)100-<i>x> with a Co volume fraction of 34≤<i>x>≤100 using co
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COMPUŞI COORDINATIVI ŞI COMPOZIŢII CU PROPRIETĂŢI UTILE PENTRU BIOTEHNOLOGII AGRICOLE
Directory of Open Access Journals (Sweden)
Ion BULHAC
2015-12-01
Full Text Available Au fost scoşi în evidenţă compuşi coordinativi ai fierului, cobaltului, manganului de tip μ3-oxo homo- şi heteronucleari, dioximaţi şi compoziţii din macro-, microelemente şi vitamine cu proprietăţi de stimulatori de creştere şi dezvoltare a plantelor de cultură, de ridicare a energiei de germinare a seminţelor tratate, antioxidante, de majorare a rezistenţei plan-telor la secetă şi temperaturi suboptimale, de mărire a productivităţii plantelor şi de îmbunătăţire a calităţii producţiei. Au fost stabilite condiţiile de utilizare, inclusiv concentraţia optimală a compuşilor coordinativi în procedeele de cultivare a plantelor agricole: porumb, sfeclă de zahăr, sfeclă roşie de masă, castraveţi, tomate, fasole, soia şi a.COORDINATION COMPOUNDS AND COMPOSITIONS WITH USEFUL PROPERTIES FOR AGRICULTURE BIOTECHNOLOGIESThere were highlighted iron, cobalt, manganese μ3-oxo homo- and heteronuclear coordination compounds, dioximates and compositions consisting of macro-, microelements and vitamins with stimulation properties of crop plants growth and development, rising the germination energy of treated seeds, antioxidant properties, increasing plant resistance to drought and suboptimal temperatures, incrementing plant productivity and improvement of production quality. There were established the terms of use, including optimal concentration of coordination compounds in the cultivation processes of agricultural plants: corn, sugar beets, beetroot, cucumbers, tomatoes, beans, soy and other.
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Bare et spil? Perspektiver i at bruge serious games i arbejdsmiljøundervisningen i erhvervsskolerne
Directory of Open Access Journals (Sweden)
Helene Meldgaard Pedersen
2016-11-01
Full Text Available Et godt psykisk arbejdsmiljø på erhvervsskole og læreplads kan være afgørende for, om elever færdiggør deres uddannelse. For at ruste elever til at håndtere problematikker på arbejdspladsen, er det vigtigt med en relevant og virkelighedsnær undervisning i psykisk arbejdsmiljø. I denne artikel undersøger vi perspektiverne i at bruge et bestemt 'serious game', der kombinerer pædagogiske og spilrelaterede elementer, i undervisningen i psykisk arbejdsmiljø på erhvervsskoler i Danmark. Undersøgelsen fokuserer specifikt på læring: hvordan elever og lærlinge tilegner sig viden om de udfordringer og dilemmaer, de kan møde på en arbejdsplads. Undervisningssitet www.ditarbejdslivnu.dk er et undervisningsmateriale til erhvervsskolernes undervisning i arbejdsmiljø, arbejdspladskultur og samarbejde. Sitet består af et Dilemmaspil, historier baseret på elevers og lærlinges arbejdserfaringer, en vidensbank samt didaktisk materiale. I artiklen inddrages data fra en afprøvning af undervisningssitet foretaget inden for fagene frisør, social- og sundhedshjælper, tømrer samt bygningsmaler. Resultaterne peger på, at eleverne gennem brug af spillet motiveres til at forholde sig aktivt og reflekterende til de emner og dilemmaer, der præsenteres, og at de identificerer sig med de problematikker, der beskrives af andre unge i de autentiske historier. Ud fra et socialkonstruktivistisk læringsperspektiv diskuterer artiklen, hvilke perspektiver der er i at bruge ’serious games’ i arbejdsmiljøundervisningen.
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Edge, Charles
2010-01-01
Are you an IT professional involved in deploying the iPhone and or iPad in your company or organization? Do you have fellow employees who are eager to use their iPhones and iPads for work? Then this is the book for you. Enterprise iPhone and iPad Administrator's Guide provides step-by-step instructions on how to deploy and integrate the iPhone within a range of professional environments - from large businesses and educational institutions to medium-sized offices. This book ensures that the process is achieved in a streamlined, efficient, and cost-effective manner. The guide begins with an intr
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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. -
Forskelle og ligheder i oplevelsen af arbejdsmiljøet i ældreplejen i ni kommuner fra 2005-2008
DEFF Research Database (Denmark)
Clausen, Thomas; Bern, Stine Hvid; Carnelro, Isabella Gomes
Formålet med nærværende rapport er at undersøge, hvordan arbejdsmiljø, helbred og trivsel udviklede sig blandt medarbejderne i ældreplejen i de ni kommuner, som deltog i de tre runder af spørgeskemaundersøgelsen Arbejde i ældreplejen, der blev gennemført i 2005, 2006 og 2008. I rapporten har vi...... sammenlignet udviklingen i arbejdsmiljø, helbred og trivsel blandt medarbejderne i de ni kommuner ved at se på udviklingen inden for følgende 10 områder: · Fysisk arbejdsmiljø · Smerter i bevægeapparatet · Krav i arbejdet · Ressourcer i arbejdet · Positive tilstande i arbejdet · Helbred og velbefindende...... · Plejekvalitet · Oplevelser af krænkende adfærd · Sygefravær · Forventninger om arbejdsophør. Undersøgelsen viser, at der inden for nogle af de analyserede områder af arbejdsmiljøet findes en relativt begrænset spredning mellem de ni kommuner, der har deltaget i de tre runder i undersøgelsen 'Arbejde i...
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“I don't use a condom (with my regular partner) because I know that I ...
African Journals Online (AJOL)
I don't use a condom (with my regular partner) because I know that I'm faithful, but with everyone else I do”: The cultural and socioeconomic determinants of sexual partner concurrency in young South Africans.
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Production of gaseous radiotracers CH3I and I2 through Na123I salt
International Nuclear Information System (INIS)
Candeiro, R.E.M.; Pereira, W.P.
2011-01-01
The objective of the present work was to develop, separately, methodology for production of two gaseous tracers through the sodium iodide NaI marked with 123 I. Found in the nature in form different, the iodine has been used in diverse works in the area of the industry and health. These two forms of the gaseous iodine, the methyl iodide, CH 3 I, and molecular iodine, I 2 , are very unstable and volatile in the ambient temperature and presents different problems in clean-up and monitoring systems. The syntheses were processed with sodium iodide (NaI) 1M aqueous solution marked with 1 23I . The production of gas I 2 was realized with in chlorine acid (HCl) and sodium iodate salt (NaIO 3 ) and the CH 3 I was used, the salt of NaI and the reagent (CH 3 ) 2 SO 4 . The production of gases was initially realized through in unit in glass with an inert material and the purpose was to study the kinetic of reaction and to determine the efficiency of production. The two synthesis occurs in the reaction bottle and after of produced, the gas is stored in the collect bottle that contains a starch solution for fixed the I 2 , and in syntheses of CH 3 I contains a silver nitrate solution for your fixation. To determine the efficiency of production of gases, analytic tests were realized, where the consumption of iodide ions of the bottle of reaction are measured. The optimization of production of the each gaseous tracer was studied varying parameter as: concentration of iodide, concentration of acid and temperature. After, the syntheses of the radiotracers were realized in the compact unit, having been used as main reagent the salt radiated of sodium iodide, Na 123 I. The transportation of elementary iodine and methyl iodine was studied by a scintillation detector NaI (2 x 2)' positioned in the reaction bottle. (author)
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Directory of Open Access Journals (Sweden)
Camarasa, Josep M.
2000-12-01
Full Text Available <i>Silene niceensisi> All. (Cariophillaceae is a plant found on litoral sands of Western Mediterranian and also of Iberian Atlantic shores from Gibraltar to the Rias Baixas and in some places in Greece. It had been described twice in prelinnean times but Linné has neglected it in his works. First prelinnean description, though published by Pierre Magnol in the Appedix to the second edition (1686 of <i>Botanicum monspeliensei>,has been wrote on the basis of materials and a short description sent to Magnol by Jaume Salvador i Pedrol. One specimen of this plant in Salvador Herbarium hold a short description that could be the original one by Jaume Salvador. The second one was published on a different name by Tournefort in <i>Institutiones Rei herbariaei>. It is also one specimen of <i>S. niceensisi>All. in Salvador Herbarium under this identity. Pourret, studying this herbarium to put on etiquets the linnean name of each specimen (1792-98, realize the specific identity of both <i>S. niceensisi> All. specimens but ignoring the description published by Allioni in 1773 he give them the new name of <i>S. arenariai> Pourr. Also Desfontaines described under the name of <i>S. arenariai> Desf. in his <i>Flora Atlanticai> (1798-99 the same species on the basis of specimens collected near the Gibraltar Strait. Both. Pourrett and Desfontaines names, or today invalid and under sinonimy.
La cariofil làcia <i>Silene niceensisi> All. pròpia dels sorrats litorals de la Mediterrània occidental i present també a les costes atlàntiques ibèriques. des de l' estret de Gibraltar fins a les Rias Baixas i en alguna localitat grega, havia estar descrita al menys dues vegades temps prelinneans malgrat que Linné mateix la ignorés a les seves obres. La primera descripció prelinneana. bé que publicada por Péire Magnol a l’apèndix a la segona edició del seu <i>Botanicum monspeliensei>,. es feu sobre la base de materials i fins i tot d -
iPhone and iPad apps for absolute beginners
Lewis, Rory
2013-01-01
So how do you build an application for the iPhone and iPad? Don't you need to spend years learning complicated programming languages? What about Objective-C and Cocoa touch? The answer is that you don't need to know any of those things! Anybody can start building simple apps for the iPhone and iPad, and this book will show you how. This update of an Apress bestseller walks you through creating your first app, using plain English and practical examples using the iOS 6 software development platform and more. It cuts through the fog of jargon and misinformation that surrounds iPhone and iPad app
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Does <i>Tamarix dalmaticai> (Tamari caceae occur in Spain?
Directory of Open Access Journals (Sweden)
Villar, José Luis
2012-12-01
Full Text Available The presence of <i>Tamarix dalmaticai> in the Iberian Peninsula, and its confusion in Spain with the native species <i>T. boveanai> and <i>T. africanai>, is discussed. A morphological comparison between these three species, and a critical study of the causes for the confusion between them, is presented. This includes an analysis of the variability in some morphological characters used for the identification of these species, together with clarifications of some discrepancies between the original descriptions and our observations of the type material. As a conclusion, <i>T. dalmaticai> is excluded from the Iberian and Balearic floras.
Se discute la presencia de <i>Tamarix dalmaticai> en la Península Ibérica e Islas Baleares. Se pone de manifiesto la frecuente confusión de esta especie, en el territorio peninsular, con las nativas <i>T. boveanai> y <i>T. africanai>. Se realiza una comparación morfológica entre las tres especies y un estudio crítico de las causas que han provocado dicha confusión. Así mismo, se discute la variabilidad existente en algunos caracteres morfológicos utilizados para la identificación de estas especies, así como algunas discrepancias entre las descripciones originales publicadas y el material tipo. Como conclusión se descarta la presencia de <i>T. dalmaticai> en la flora ibero-balear. -
<i>Plesiomonas shigelloidesi>: um enteropatógeno emergente?
Directory of Open Access Journals (Sweden)
J. P. FALCãO
2009-01-01
Full Text Available
<i>Plesiomonas shigelloidesi> é um bacilo Gram-negativo, pertencente à família <i>Enterobacteriaceae>, isolado de água doce e salgada, de peixes de água doce, mariscos e de inúmeros tipos de animais. Suspeita-se que a maioria das infecções humanas causadas por <i>P. shigelloidesi>, seja veiculada pela água, pois a bactéria está presente em águas não tratadas que são usadas para beber, águas recreacionais ou água para lavar alimentos que são consumidos sem cozimento ou aquecimento. A ingestão de <i>P. shigelloidesi> não causa sempre doença no animal hospedeiro, mas o microrganismo pode permanecer temporariamente como membro transitório não infeccioso da microbiota intestinal. A bactéria é isolada de fezes de pacientes com diarréia, mas algumas vezes também de fezes de indivíduos sem sintomas. A doença causada por <i>P. shigelloidesi> é a gastrenterite, que normalmente é auto-limitante, com febre, calafrio, dor abdominal, náusea, diarréia ou vômito. Em casos graves, as fezes diarréicas podem ser verde-amareladas, espumosas e com presença de sangue. A bactéria pode também causar infecções extra-intestinais. Ademais, pode produzir toxinas e ser invasora. As características utilizadas para considerar <i>P. shigelloidesi> como um enteropatógeno não são totalmente convincentes. Embora seja isolada de pacientes com diarréia e incriminada em vários surtos epidêmicos envolvendo água e alimentos contaminados, não foi possível identificar em muitas amostras de <i>P. shigelloidesi>, associadas com infecções gastrintestinais, um mecanismo de virulência definitivo. Palavras-chave: <i>P. shigelloidesi>; enteropatógeno; gastrenterite; diarréia; infecções extra-intestinais.
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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
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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 -
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...
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iPhone'i peab saama lukust lahti teha / Dannar Leitmaa
Leitmaa, Dannar, 1982-
2008-01-01
Kuigi Euroopa Liidu direktiivi kohaselt peab mobiiltelefoni omanikul olema võimalus telefoni lukustusest avada, näeb iPhone'i tootva Apple'i ärimudel ette iPhone'i ostmist ühelt operaatorilt. iPhone 3G telefonide ametlik maaletooja Eestis on EMT, kes pakub neid müügiks kahes erinevas hinnapaketis. Lisa: Juba öösel seisti pikas järjekorras
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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
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<i>Memorias de Adrianoi>: Un modelo de excelencia
Directory of Open Access Journals (Sweden)
Espinosa, Luciano
2006-12-01
Full Text Available This book (Marguerite Yourcenar, 1951 is much more than a historical novel: of course there are Literature (fictions, but also History (facts and above all a great reflection about the art of living, when the man was alone and without gods (Flaubert <i>dixit>. The Emperor tells us how he builds himself –<i>operari est essei>- upon the unity of Ethics and Aesthetics, with a combination of knowledge, discipline and hedonism. This paper shows the keys of his personal and political project, around the Greek ideas (<i>Humanitas, Felicitas, Libertasi> and the Roman exercise of power. And that includes bodies, feelings and actions like concepts from the point of view of every day life.
Este libro (Marguerite Yourcenar, 1951 es mucho más que una novela histórica: por supuesto hay literatura, pero también historia y sobre todo una gran reflexión sobre el arte de vivir, en un tiempo en que el hombre estaba solo y sin dioses (Flaubert <i>dixit>. El Emperador nos cuenta cómo se construye a sí mismo –<i>operari est essei>-, a partir de la unidad de ética y estética, con una combinación de conocimiento, disciplina y hedonismo. Este ensayo muestra las claves de su proyecto personal y político, en torno a las ideas griegas (<i>Humanitas, Felicitas, Libertasi> y al ejercicio del poder romano. Lo que incluye a los cuerpos, los afectos y las acciones como conceptos desde el punto de vista de la vida cotidiana.
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Determination of Sc and Th in ScI3-NaI-ThI4 sample
International Nuclear Information System (INIS)
Yuan Hui; Yang Zhihong
1999-01-01
Determination of Sc and Th in ScI 3 -NaI-ThI 4 sample is studied by X-ray fluorescence and complexometry. The effect of working condition of Sc on X-ray fluorescence spectrometer and different sample-making method on the determination of Sc and Th are studied experimentally. The X-ray fluorescence analysis of determination of Sc and Th is developed by polyester film sample-making technique, the measuring precision of Sc and Th is better than 4%. The effect of acidity and temperature on the determining end point of titration is obvious. The results of different complexometric methods are compared. The precision of Sc and Th is less than 2% by comlexometry, it is fit for the routine analysis of ScI 3 -NaI-ThI 4 sample
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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...
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Obiteljska kohezivnost i zadovoljstvo životom djevojaka i mladića iz sela i iz grada
Bezinović, Petar; Manestar, Kristina; Ristić Dedić, Zrinka
2009-01-01
Predmet rada jest obiteljska kohezivnost u seoskim i gradskim obiteljima, analizirana temeljem rezultata pregledna istraživanja (survey research) provedena na proporcionalnom uzorku srednjoškolaca (4.865 adolescenata) u Primorsko-goranskoj, Ličko-senjskoj i Dubrovačko-neretvanskoj županiji. Ispitivanje je provedeno primjenom prilagođene ljestvice obiteljskih rituala i zajedničkih aktivnosti (Compan i sur., 2002). Rezultati upućuju na značajne razlike seoskih i gradskih obitelji u neki...
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2010-08-18
... DEPARTMENT OF HOMELAND SECURITY U.S. Citizenship and Immigration Services Agency Information... of Homeland Security, U.S. Citizenship and Immigration Services (USCIS) will be submitting the...: Form I-864, Form I-864A, Form I-864EZ, and Form I-864W; U.S. Citizenship and Immigration Services...
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Estudos cromossómicos em <i>Pteridophyta> de Portugal, III
Directory of Open Access Journals (Sweden)
Queirós, Margarida
1997-12-01
Full Text Available Chromosome numbers of nine taxa of <i>Pteridophyta> from Portugal are reported. Earlier counts of seven of these taxa have been carried out on material from other Portuguese places. <i>Polypodium vulgarei> L. and <i>P. interjectumi> Shivas is now first studied under the caryological point of view.
Se ha estudiado el número cromosómico gamético de nueve táxones de <i>Pteridophyta> portugueses, siete de los cuales coinciden con los datos indicados anteriormente para otras localidades del país. Se dan a conocer por primera vez, en material portugués, los siguientes: <i>Polypodium vulgarei> L. y <i>P. Interjectumi> Shivas. -
iBooks and ePeriodicals on the iPad
Biersdorfer, J
2010-01-01
This Mini Missing Manual takes you down the virtual rows of Apple's iBookstore, to help you find, buy, and download books and subscribe to magazine and newspapers on your iPad. You'll learn now to navigate your books and periodicals and sync them back to iTunes to swap them on and off your iPad to free up storage space. In addition, you'll find out where to get free books in the iBookstore, and where you can shop for iPad-compatible books, both free and for-pay, outside of Apple's domain. Finally, you'll learn the fine art of eBook navigation, including how to change a book's font and font
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Energy Technology Data Exchange (ETDEWEB)
Vignesh, Kuduva R. [IITB-Monash Research Academy, IIT Bombay, Powai, Mumbai (India); Langley, Stuart K. [School of Science and the Environment, Division of Chemistry, Manchester Metropolitan University, Manchester (United Kingdom); Swain, Abinash; Rajaraman, Gopalan [Department of Chemistry, Indian Institute of Technology Bombay, Powai, Mumbai (India); Moubaraki, Boujemaa; Murray, Keith S. [School of Chemistry, Monash University, Clayton, VIC (Australia); Damjanovic, Marko; Wernsdorfer, Wolfgang [Institute Neel, CNRS, Universite Grenoble Alpes, Grenoble (France); Institute of Nanotechnology (INT), Karlsruhe Institute of Technology (KIT), Eggenstein-Leopoldshafen (Germany)
2018-01-15
The synthesis, magnetic properties, and theoretical studies of three heterometallic {Cr"I"I"ILn"I"I"I_6} (Ln=Tb, Ho, Er) complexes, each containing a metal topology consisting of two Ln{sub 3} triangles connected via a Cr{sup III} linker, are reported. The {CrTb_6} and {CrEr_6} analogues display slow relaxation of magnetization in a 3000 Oe static magnetic field. Single-crystal measurements reveal opening up of the hysteresis loop for {CrTb_6} and {CrHo_6} molecules at low temperatures. Ab initio calculations predict toroidal magnetic moments in the two Ln{sub 3} triangles, which are found to couple, stabilizing a con-rotating ferrotoroidal ground state in Tb and Ho examples and extend the possibility of observing toroidal behaviour in non Dy{sup III} complexes for the first time. (copyright 2018 Wiley-VCH Verlag GmbH and Co. KGaA, Weinheim)
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Anderson, Daniel
2010-01-01
I'm talking about the ways we represent ourselves and our world. I've put some thoughts on the topic together here--a gathering that enacts new media creating and takes up conceptual layers like metaphors, models, and composing. The primary sources are videos from the Get a Mac campaign, aka I'm a Mac; I'm a PC ads. Posthuman concepts blending…
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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...
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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.
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En reise i tid og landskap – Planlegging og tilrettelegging i kulturlandskapet, Valle i Setesdal
Hillestad, Ingunn
2010-01-01
Oppgaven omhandler hvordan fremtidig utvikling i Valle i Setesdal kan legges tilrette gjennom lansdskapsplanlegging. Selve initiativet kom fra Valle kommune, men jeg har stått fritt i forhold til vinklingen av oppgaven. Målet er å aktivisere Nomeland, et sentrumsnært område og omkringliggende steder. Planleggingen skal rettes mot friluftsliv og kultur. Et annet mål er å få en bred og sammensatt brukergruppe, med forankring i lokalbefolkningen og et tilbud for turister. Oppgaven er strukturert...
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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
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Directory of Open Access Journals (Sweden)
Niño Nancy Eunice
2008-04-01
Full Text Available
Este estudio se realizó en el Centro de Investigaciones y Asesorías Agroindustriales de la Universidad de Bogotá Jorge Tadeo Lozano, Chía (Cundinamarca, bajo condiciones de invernadero, durante diez meses de cultivo. Se evaluó el efecto de diferentes densidades poblacionales de <i>Meloidogyne haplai> (0, 50, 100, 200, 500 y 1.000 larvas J2/100 cc de suelo, sobre parámetros productivos de la uchuva: altura de las plantas, diámetro de tallo, peso fresco, peso seco y biomasa de follaje, peso fresco de raíces, índice de nodulación radicular y peso fresco de frutos. Se hizo análisis de varianza, pruebas de contrastes y prueba de Tukey (α=0,05, en un diseño completamente aleatorio, con seis tratamientos y cinco repeticiones, mediante el programa SAS v.8.0. El tratamiento de 500 larvas J2/100 cc suelo fue el que más afectó la altura de las plantas, el diámetro del tallo, la biomasa de follaje, el peso fresco de raíces y presentó el índice de nodulación radical más alto. No se encontraron diferencias significativas entre los tratamientos inoculados con <i>M. haplai> , para peso fresco, peso seco de follaje y peso fresco de frutos, ni para el número de larvas J2 en suelo y masas de huevos. Mediante la ecuación: Y = m + (1–mZP-T propuesta por Seinhorst (1965, se estableció un límite de tolerancia de 50 larvas J2 de <i>M. haplai> /100 cc suelo, para peso fresco de raíces y un límite de tolerancia de 450 larvas J2 de <i>M. haplai> /100 cc suelo, para biomasa de follaje.
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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)
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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
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Energy Technology Data Exchange (ETDEWEB)
Catanoso, Marcela Forli
2011-07-01
The {sup 123}I and {sup 131}I are iodine radioisotopes widely used in Nuclear Medicine. The radioisotope {sup 123}I is used in diagnosis through the SPECT technique and is routinely produced at IPEN in cyclotron through the reaction: '1{sup 24}Xe (p, 2n) '1{sup 23}Cs -> {sup 123}Xe -> {sup 123}I. The radioisotope {sup 131}I is used both in diagnosis and therapy due to its physical characteristics of decay by {beta}{sup -} and its {gamma}-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: {sup 130}Te (n, {gamma}) ->{sup 131}Te -> {sup 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 {sup 123}I and {sup 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{sup 23}I e {sup 131}I radionuclidic purity using a hyper pure germanium detector, chemical purity using ICP-OES and the retention and elution study of {sup 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 {sup 131}I samples and Te, Tc e Co on {sup 123}I samples. The chemical purity analyses showed the presence of Al and Mo in {sup 123}I, coming from the window material of the target holder and the presence of Al and Te in {sup
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Mindfulness i skolen : ABC, 123, samt elever i balanse
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.
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Search for a pentaquark decaying to <i>pK>0S in <i>γN>
Energy Technology Data Exchange (ETDEWEB)
Link, J. M.; Yager, P. M.; Anjos, J. C.; Bediaga, I.; Castromonte, C.; Machado, A. A.; Magnin, J.; Massafferri, A.; de Miranda, J. M.; Pepe, I. M.; Polycarpo, E.; dos Reis, A. C.; Carrillo, S.; Casimiro, E.; Cuautle, E.; Sánchez-Hernández, A.; Uribe, C.; Vázquez, F.; Agostino, L.; Cinquini, L.; Cumalat, J. P.; Frisullo, V.; O' Reilly, B.; Segoni, I.; Stenson, K.; Butler, J. N.; Cheung, H. W. K.; Chiodini, G.; Gaines, I.; Garbincius, P. H.; Garren, L. A.; Gottschalk, E.; Kasper, P. H.; Kreymer, A. E.; Kutschke, R.; Wang, M.; Benussi, L.; Bertani, M.; Bianco, S.; Fabbri, F. L.; Pacetti, S.; Zallo, A.; Reyes, M.; Cawlfield, C.; Kim, D. Y.; Rahimi, A.; Wiss, J.; Gardner, R.; Kryemadhi, A.; Chung, Y. S.; Kang, J. S.; Ko, B. R.; Kwak, J. W.; Lee, K. B.; Cho, K.; Park, H.; Alimonti, G.; Barberis, S.; Boschini, M.; Cerutti, A.; D' Angelo, P.; DiCorato, M.; Dini, P.; Edera, L.; Erba, S.; Inzani, P.; Leveraro, F.; Malvezzi, S.; Menasce, D.; Mezzadri, M.; Moroni, L.; Pedrini, D.; Pontoglio, C.; Prelz, F.; Rovere, M.; Sala, S.; Davenport, T. F.; Arena, V.; Boca, G.; Bonomi, G.; Gianini, G.; Liguori, G.; Lopes Pegna, D.; Merlo, M. M.; Pantea, D.; Ratti, S. P.; Riccardi, C.; Vitulo, P.; Göbel, C.; Olatora, J.; Hernandez, H.; Lopez, A. M.; Mendez, H.; Paris, A.; Quinones, J.; Ramirez, J. E.; Zhang, Y.; Wilson, J. R.; Handler, T.; Mitchell, R.; Engh, D.; Givens, K. M.; Hosack, M.; Johns, W. E.; Luiggi, E.; Nehring, M.; Sheldon, P. D.; Vaandering, E. W.; Webster, M.; Sheaff, M.
2006-08-01
We present a search for a pentaquark decaying strongly to <i>pK>0S in <i>γN> collisions at a center-of-mass energy up to 25 GeV/c2. Finding no evidence for such a state in the mass range of 1470 MeV/c2 to 2200 MeV/c2, we set limits on the yield and on the cross section times branching ratio relative to Σ* (1385)± and K* (892) +.
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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?...
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Directory of Open Access Journals (Sweden)
Cantor Fernando
2009-12-01
Full Text Available
Trabajos preliminares realizados en cultivos comerciales de rosa en la Sabana de Bogotá han permitido evidenciar la presencia de avispas del género <i> Praoni> parasitando áfidos. Con el fin de explorar su potencial como controlador biológico, el presente estudio caracterizó la biología y el ciclo reproductivo de <i>Praon> sp. en condiciones de laboratorio bajo seis temperaturas constantes: 15, 17, 20, 25, 28 y 30°C. Áfidos parasitados fueron mantenidos en incubadoras a la temperatura deseada con 12 horas luz y 12 horas de oscuridad. Diariamente se realizaron disecciones de los áfidos parasitados para determinar el estado de desarrollo del parasitoide, lo que hizo posible determinar la duración promedio en días de cada estado, desde huevo hasta adulto. A todas las temperaturas, excepto a 15°C, se observó un desarrollo completo de los diferentes estadios, para los cuales se ajustaron modelos de tasa de desarrollo en función de la temperatura. El estado de huevo presentó el mayor umbral de temperatura mínima requerida para completar su desarrollo. Tanto hembras como machos son más longevos a temperaturas bajas. A temperaturas de 17 y 20°C se encontró mayor número de machos, mientras que a 25, 28 y 30°C se halló mayor número de hembras.
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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 ...
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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....
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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....
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El <i>Scylitzes> de la Biblioteca Nacional de Madrid
Directory of Open Access Journals (Sweden)
Fernández Pomar, José María
1964-12-01
Full Text Available Not available.
El códice <i> Matritensis Vitr. 26-2i>, conocido comúnmente con el nombre de <i>Scylitzes>, es uno de los más valiosos que posee la Biblioteca Nacional de Madrid y en la actualidad es conocido en todo el mundo. La causa de su fama radica no tanto en el texto que contiene, el cual se encuentra también en otros códices de otras bibliotecas, sino en la gran riqueza de su iluminación, razón por la cual se han hecho, y siguen haciéndose, gran número de reproducciones de sus miniaturas. Este manuscrito estuvo expuesto en otro tiempo en una vitrina, cuya signatura (<i>Vitr. 26-2i> sigue conservando todavía; con anterioridad había estado colocado en el armario N y se lo conocía con la signatura <i>N-2i>. -
Energy Technology Data Exchange (ETDEWEB)
Chirgadze, Yuri N. [Institute of Protein Research, Russian Academy of Sciences, Pushchino 142290, Moscow Region, Russia; Boshkova, Eugenia A. [Institute of Protein Research, Russian Academy of Sciences, Pushchino 142290, Moscow Region, Russia; Battaile, Kevin P. [Advanced Photon Source, Argonne National Laboratory, Hauptman–Woodward Medical Research Institute, IMCA-CAT, Argonne, IL 60439, USA; Mendes, Vitor G. [Department of Biochemistry, University of Cambridge, Cambridge CB2 1GA, UK; Lam, Robert [Campbell Family Cancer Research Institute, Ontario Cancer Institute, Princess Margaret Hospital, University Health Network, Toronto, Ontario M5G 2C4, Canada; Chan, Tiffany S. Y. [Campbell Family Cancer Research Institute, Ontario Cancer Institute, Princess Margaret Hospital, University Health Network, Toronto, Ontario M5G 2C4, Canada; Romanov, Vladimir [Campbell Family Cancer Research Institute, Ontario Cancer Institute, Princess Margaret Hospital, University Health Network, Toronto, Ontario M5G 2C4, Canada; Pai, Emil F. [Campbell Family Cancer Research Institute, Ontario Cancer Institute, Princess Margaret Hospital, University Health Network, Toronto, Ontario M5G 2C4, Canada; Department of Biochemistry, University of Toronto, Toronto, Ontario M5S 1A8, Canada; Department of Molecular Genetics, University of Toronto, Toronto, Ontario M5S 1A8, Canada; Department of Medical Biophysics, University of Toronto, Toronto, Ontario M5S 1A8, Canada; Chirgadze, Nickolay Y. [Campbell Family Cancer Research Institute, Ontario Cancer Institute, Princess Margaret Hospital, University Health Network, Toronto, Ontario M5G 2C4, Canada; Department of Pharmacology and Toxicology, University of Toronto, Toronto, Ontario M5S 1A8, Canada; X-CHIP Technologies Inc., Toronto, Ontario, Canada
2017-01-16
The crystal structures of protein SA0856 from Staphylococcus aureus in its apo-form and in complex with a Zn2+-ion have been presented. The 152 amino acid protein consists of two similar domains with α + β topology. In both crystalline state and in solution, the protein forms a dimer with monomers related by a twofold pseudo-symmetry rotation axis. A sequence homology search identified the protein as a member of the structural family Glyoxalase I. We have shown that the enzyme possesses glyoxalase I activity in the presence of Zn2+, Mg2+, Ni2+, and Co2+, in this order of preference. Sequence and structure comparisons revealed that human glyoxalase I should be assigned to a subfamily A, while S. aureus glyoxalase I represents a new subfamily B, which includes also proteins from other bacteria. Both subfamilies have a similar protein chain fold but rather diverse sequences. The active sites of human and staphylococcus glyoxalases I are also different: the former contains one Zn-ion per chain; the latter incorporates two of these ions. In the active site of SA0856, the first Zn-ion is well coordinated by His58, Glu60 from basic molecule and Glu40*, His44* from adjacent symmetry-related molecule. The second Zn3-ion is coordinated only by residue His143 from protein molecule and one acetate ion. We suggest that only single Zn1-ion plays the role of catalytic center. The newly found differences between the two subfamilies could guide the design of new drugs against S. aureus, an important pathogenic micro-organism.
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Pilone, Dan
2011-01-01
Let's say you have a killer app idea for iPhone and iPad. Where do you begin? Head First iPhone and iPad Development will help you get your first application up and running in no time. You'll not only learn how to design for Apple's devices, you'll also master the iPhone SDK tools -- including Interface Builder, Xcode, and Objective-C programming principles -- to make your app stand out. Whether you're a seasoned Mac developer who wants to jump into the App store, or someone with strong object-oriented programming skills but no Mac experience, this book is a complete learning experience for
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Sygehusene i den islamiske verden i den klassiske tid
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)
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Directory of Open Access Journals (Sweden)
López Alfredo
2007-06-01
Full Text Available
Se ha demostrado que la transformación de papa (<i>Solanum tuberosumi> mediada por <i>Agrobacterium tumefaciensi> es dependiente del genotipo y que la mayoría de protocolos de transformación reportados son ineficientes al aplicarlos en la subespecie <i>andigena>. En esta propuesta se manejaron los procesos iniciales de mejoramiento genético de la nueva variedad colombiana de papa Pastusa suprema (<i>Solanum tuberosum sp. andigenai> que es altamente androestéril, característica de gran importancia para los organismos modificados genéticamente. Esta variedad resultó de la hibridación interespecífica de tres especies de papa (<i>Solanum stoloniferumi>, <i>Solanum phurejai> var. Yema de huevo y <i>Solanum tuberosum sp. andigenai> var. Parda pastusa. Se transformaron explantes internodales mediante el vector pCambia2301 que posee un gen reportero de la β-glucoronidasa y un gen de resistencia a la kanamicina. Se obtuvo un porcentaje de transformación inicial de 31 ± 2,5%, que se expresó mediante formación de callo sobre medios de selección y una frecuencia final con base en el ensayo GUS de 30%. Este es el primer reporte de transformación de un híbrido interespecífico de tres especies diferentes.
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Tumor therapy with 125I-octreotide and 125I-UdR
International Nuclear Information System (INIS)
Fan, W.; Zhu, R.; Yang, C.; Sun, J.J.; Xu, Y.J.; Zhang, Y.J.; Wu, M.J.; Wang, D.J.
2005-01-01
Purpose: To determine the tumor cell damage effect with Auger-electron emitter 125 I in different chemical states. Methods: (1) [Tyr 3 ] octreotide (TOC) and UdR are labeled with 125 I,respectively. (2) Receptor analysis of 125 I-TOC on small cell lung cancer (SCLC) NCI-H446 cell lines is performed comparing with normal lymph cells. NCI-H446 cells added various dose of 125 I-TOC are incubated for different time with 125 I-Nal and non-labeled TOC as control. The capacity of NCI-H446 cell lines bound and internalization of 125 I TOC are determined. The radiation damage of tumor cells is measured by MTF methods. (3) The killing effects of 125 I-UdR in human pancreatic cancer cell line Bax-Pc and Sca-BER bladder carcinoma cells are evaluated with the similar methods. I-UdR penetrating into the Sca-BER cell nucleus is observed with confocal microscope. The grow suppression and clonogenic formation of Sca-BER cells after incubation with 125 I-UdR are analyzed. Proliferation fraction and S phase cell fraction of Sca-BER cell added 125 I-UdR is measured with flow cytometric analysis. Results: (1) Kd=(0.56∼2.0) x 10 -11 mol/L and B max =(1.17∼2.0) x 10 5 cell site are obtained by receptor analysis of 125 I-TOC on NCI-H446 cells. Comparatively, the difference between total binding and non-specific binding is low and there is no saturation of specific binding for normal lymphocyte. About 50% of 125 I-TOC is internalized into the NCI-H446 cell nucleus at 24h after incubation. The damige of NCI-H446 cells by 125 I-TOC is clearly observed. (2) The penetration amount of 125 I-UdR into cell nucleus increases with the incubate time when the concentration of 125 I-UdR is in the range of 10∼500 kBq/mL and reaches the peak fraction of 94% at 36 h after incubation. The radioactivity of 125 I-UdR is then achieved equelibration and no more increased with time. The linear correlation with γ=0.867∼0.978 between the concentration of 125 I-UdR in cell nucleus and the incubation time
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Diel, Dirnete; Lagranha, Valeska Lizzi; Schuh, Roselena Silvestri; Bruxel, Fernanda; Matte, Ursula; Teixeira, Helder Ferreira
2018-01-01
Mucopolysaccharidosis type I (MPS I) is an autosomal recessive disease caused by deficiency of α-l-iduronidase (IDUA), which results in the lysosomal accumulation of glycosaminoglycans (GAG) leading to widespread clinical manifestations. The microencapsulation of IDUA overexpressing recombinant cells has been considered as a promising strategy for the treatment of MPS I. This study aimed at the optimization of alginate microcapsules containing recombinant BHK (Baby Hamster Kidney) cells (rBHK) overexpressing IDUA produced by electrostatic extrusion technique. The alginate microcapsule (MC-A) optimization study was carried out by means of an experimental Box-Behnken Design that allowed the simultaneous evaluation of the influence of voltage (kV), alginate/cell suspension flow (mL/h), and alginate concentration (%) on size and IDUA activity. The optimal conditions of voltage (10kV), flow (25mL/h), and alginate concentration (1.3%) made possible to obtain the smallest microcapsules showing the highest IDUA activity. After optimization, the microcapsules were sequentially coated with PLL and alginate (MC-APA) to increase their stability. MC-A and MC-APA presented monodisperse populations (span<1.22) with an average diameter of less than 350μm. The coating increased the mechanical stability of MC-APA by about 6-fold and modulated the permeability to the enzyme. Surface analyzes of MC-APA showed the presence of PLL bands, suggesting that the last alginate layer appears to have only partially coated the PLL. After 30days of subcutaneous implantation of the MC-APA microcapsules containing rBHK cells in a MPS I murine model, a significant increase in IDUA activity was observed in the skin near the implant. Histological analysis revealed an inflammatory infiltrate at the application site, which did not prevent the release of the enzyme under the conditions evaluated. Taken together, the overall results demonstrate the feasibility of MC-APA as a potential alternative for
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iPad for the older and wiser get up and running with your Apple iPad, iPad Air and iPad Mini
McManus, Sean
2013-01-01
Apple's iPad puts a limitless world of entertainment, communication, and everyday functionality in the palms of your hands. iPad for the Older and Wiser, 4th Edition quickly teaches you how to make the most of your new gadget with easy-to-follow instructions. Fully updated to include the iPad Air and iOS 7, this step-by-step guide shows you exactly how to set up your device and discover what it can do - all in jargon-free language. Learn how to: Set up your iPad and copy music and photos from your computerKeep in touch with email and messagesBrowse the web and shop onlineHave video chats wi
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Storage quality in different brines of pickled capers (<i>Capparis> spp.
Directory of Open Access Journals (Sweden)
Özcan, Musa
1999-08-01
Full Text Available Middle sized buds of <i>Capparis spinosai> L. var. <i>spinosa> and <i>Capparis ovatai> Desf. var. <i>canescens> (Coss. Heywood collected from wild plants were pickled for two months. The buds after the fermentation were processed in 10% and 20% brines. Storage stability of the fermented capers was determined by physical, chemical and microbiological analysis at certain interval in 10 and 20% old or fresh brines 180 days. Storaged in 10% and 20% old or fresh brines for 180 days of pickled buds of both species maintained the product quality in all samples. Acidity was higher in old brine during storage. Lactic acid bacteria (LAB growth was observed only in 10% fresh brine, it decreased from initial and 30 days at C. <i>ovata> and C. <i>spinosa>, respectively. C. <i>spinosa>, compared with C. <i>ovata> was desirable due to low sediment and more firm texture. During storage of buds in fresh brines, sediment and off-flavour were not observed for both species. Pickled products can be stored in fresh brine long-term containing at least 10% salt concentration.
Se encurtieron durante dos meses botones florales de tamaño intermedio de <i>Capparis spinosai> L. var. <i>spinosa> y <i>Capparis ovatai> Desf. var. <i>canescens> (Coss recogidos de plantas silvestres. Las alcaparras después de la fermentación se conservaron en salmueras del 10% y 20%. Las alcaparras fermentadas fueron mantenidas durante 180 días en salmueras usadas o recién preparadas (frescas del 10 y 20%, determinándose a intervalos la estabilidad durante la conservación por análisis físicos, químicos y microbiológicos. La calidad del producto se mantuvo en todas las muestras de alcaparras fermentadas durante los 180 días, tanto en 10% como en 20% y en salmueras usadas o frescas. La acidez durante la conservación fue mayor en salmueras usadas. El crecimiento de bacterias del ácido láctico se observó sólo en salmueras frescas del 10 -
Overvågning af influenza A virus i svin i 2014
DEFF Research Database (Denmark)
Krog, Jesper Schak; Hjulsager, Charlotte Kristiane; Larsen, Lars Erik
sekvensanalyse for at bestemme subtypen. Disse analyser viste, at de to mest almindelige subtyper i danske svin i 2014 var den danske variant af H1N2 og H1N1pdm09. Prævalensen af det almindelige svineinfluenza virus ”avian-like swine” H1N1 subtype er faldet drastisk og forekom i 2014 tilsyneladende mindre...... hyppigt end H1N1pdm09 subtypen. Influenzavirus af subtypen H3N2, der har cirkuleret i Danmark siden 1990, blev påvist i en enkelt indsendelse i 2014, hvilket også var tilfældet i 2013. Den centraleuropæiske variant af H1N2, der har et human-like HA gen, er i lighed med tidligere år ikke påvist i danske...... 2014 skyldes øget smitteoverførsel fra mennesker til svin. Resultaterne af overvågningen i 2014 underbygger antagelsen om at de nye reassortments fra de foregående år: H1N2hu, H1pdmN2hu og H1pdmN2sw, nu er fast etableret i de danske svinebesætninger. Ud over disse subtyper blev der i 2014 fundet en...
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Diagnosis of pheochromocytoma using (123I)-compared with (131I)-metaiodobenzylguanidine scintigraphy
International Nuclear Information System (INIS)
Furuta, Nozomu; Kiyota, Hiroshi; Yoshigoe, Fukuo; Hasegawa, Norio; Ohishi, Yukihiko
1999-01-01
Patient with pheochromocytoma (PCT) cannot be cured without operation, therefore, preoperative determination of the localization of PCT should be performed accurately. ( 131 I)-Metaiodobenzylguanidine (MIBG) scintigraphy is a gold standard for the diagnosis of PCT. However, ( 123 I)-MIBG is also found to accumulate in PCT. In order to clarify the usefulness of ( 123 I)-MIBG scintigraphy for the local detection of PCT, we compared the distribution of ( 123 I)- and ( 131 I)-MIBG in patients with or without PCT. ( 131 I)- and ( 123 I)-MIBG scintigraphy was performed in 29 and 16 patients, respectively. In the former group, 14 patients had PCT, 12 had hypertension without any adrenal disorder and three had other diseases. In the latter group, eight patients had PCT, two had hypertension without any adrenal disorder and six had other diseases. The sensitivity, specificity and accuracy of ( 123 I)- with ( 131 I)-MIBG scintigraphy were compared. The sensitivity of ( 131 I)- and ( 123 I)-MIBG scintigraphy was 85.7 and 90%, respectively. The specificity of each test was 100%. The accuracy of ( 131 I)- and ( 123 I)-MIBG scintigraphy was 93.1 and 95%, respectively. The quality of images obtained using ( 123 I)-MIBG was better than with ( 131 I)-MIBG, because ( 123 I)-MIBG generated a higher dose of γ-rays with a higher specificity than ( 131 I)-MIBG. In addition, normal adrenal grands were visualized in 50% of patients tested with ( 123 I)-MIBG scintigraphy. These results indicate that ( 123 I)-MIBG scintigraphy is a valuable tool for the local detection of PCT, as is ( 131 I)-MIBG scintigraphy. Furthermore, it is possible that ( 123 I)-MIBG can be used as an alternative to ( 131 I)-MIBG for the detection of PCT. Our study was not a prospective study and the background of the patients was not matched. Further prospective studies are needed in order to determine the efficacy of ( 123 I)-MIBG scintigraphy for the diagnosis of PCT. (author)
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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...
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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.
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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....
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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....
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Seed morphology of Iberian species of the genus <i>Aconitum> L.
Directory of Open Access Journals (Sweden)
Molero, Julià
1990-12-01
Full Text Available A biometric-morphological study of' the seeds of the taxons of the genus <i>Aconitum> L. occurring in the Iberian peninsula is given. The following features are treated: 1 arrangement of the seeds on the follicle, 2 definition of the parameters (general shape and ornamentation of the episperm, 3 biometry, and 4 description of the seeds of the Iberian taxa. Microcharacteristics related to the seed coat, shape and ornamentation or the insertion base (hilum and surrounding cells have been found most useful in differentiating the taxa; furthermore, they may provide more phylogenetic information than other features. The results given agree generally with those reported by other authors (Seitz, 1969; Cappeleti & Poldini, 1984 but some important discrepancies have been noted with reference to seminal morphology, particularly episperm ornamentation in the species A. <i>anthora. A. napellusi> s.l., <i>A. burnatiii> and <i>A. vulpariai> subsp. <i>ranunculifolium>. Seminal polymorphism is frequently observed in different geographically isolated colonies or the same species or subspecies. both in the Iberian Peninsula and in Central and Southern Europe. We conclude that polymorphism is further evidence of the phenotypical flexibility of this genus, probably owing to genetic drift in the populations. This flexibility is particularly important in colonizing species or wide ecological valence (<i>A . napellusi>, s. l.; <i>A. vulpariai> s.l..
[ca] Es presenta un estudi morfològica-biomètric de les granes dels tàxons del gènere <i>Aconitum> L. presents a la Península Ibèrica. Es tracten aspectes que fan referència a: 1 disposició de les granes al fol-licle. 2 definició de paràmetres (forma general i ornamentació de l'episperma, 3 biometria i 4 descripció de les granes dels representants ibèrics. Els microcaràcters relacionats fonamentalment amb el relleu epispèrmic, forma i ornamentació de la base d'inserció (hílum i -
Using iPhones, iPads, and iPods a practical guide for librarians
Connolly, Matthew
2014-01-01
Using iPhones and iPads: A Practical Guide for Librarians offers library professionals a clear path to Apple readiness. The authors combine their experience in library public services and mobile technology to provide easy-to-follow, step-by-step instructions to help you get up to speed.
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Archaeometric characterization of <i>Terra Sigillata Hispanicai> from Granada workshops
Directory of Open Access Journals (Sweden)
Aranda, M. A. G.
2010-04-01
Full Text Available <i>Terra Sigillatai> was a Roman pottery, which was also produced in several workshops in Hispania. Two known workshops at Granada (Cartuja and Carmen de la Muralla, or Albayzin are known to have produced both typical <i>Terra Sigillatai> and low-gloss coating related pottery. Potsherds from these workshops have been thoroughly studied from an archaeological approach, but not by analytical chemistry techniques. Here, we report a full characterization of nine selected samples from these workshops and also a tenth sample, <i>Terra Sigillatai> made in the Gaul, for comparison. The pastes characterization includes elemental analysis from XRF and quantitative mineralogical analysis by Rietveld analysis of XRPD data. Cluster analysis to both types of data has been carried out. SEM-EDX and GI-XRPD have been used to characterize the slips of the pottery. The elemental analysis results for the pastes suggest that <i>terra sigillatai> potsherds from both workshops were likely made from the same clay, different to that used to make the low-gloss coating pottery. The firing temperatures have been estimated from the phase assemblages being about 900-950ºC for the Granada <i>sigillata>.
La <i>Terra Sigillatai> fue una cerámica romana, que se produjo también en varios talleres en Hispania. Los talleres ubicados en Granada (Cartuja y Carmen de la Muralla, o Albayzín produjeron <i>Terra Sigillatai> y cerámicas de barniz mate. Ambos talleres han sido ampliamente estudiados desde un punto de vista arqueológico, pero no mediante técnicas analíticas. En este trabajo, se estudian nueve muestras de estas producciones, y una décima de <i>Terra Sigillatai> producida en la Galia, para su comparación. La caracterización de las pastas incluye análisis elemental por XRF, y análisis mineralógico cuantitativo mediante análisis de Rietveld de los datos de XRPD. Estos dos conjuntos de datos también se han evaluados mediante análisis de -
Directory of Open Access Journals (Sweden)
Cortés J.
2009-09-01
Full Text Available Se describen los nemátodos encontrados en el sistema digestivo de las especies de pargo <i>Lutjanus synagrisi> y <i>Lutjanus analisi>. Esta investigación se orientó principalmente a la clasificación taxonómica de los parásitos y no a los efectos o patologías que estos puedan ocasionar en los peces. Se tuvo en cuenta que en Colombia los pargos constituyen uno de los recursos de mayor importancia para la comercialización; <i>Lutjanus synagrisi> y <i>Lutjanus analisi> son especies demersales explotadas a lo largo de toda la región costera e insular del Caribe colombiano. Los tractos digestivos se colectaron de los pargos capturados artesanalmente (nasa, cordel por pescadores de las zonas de Santa Marta y Neguanje; se identificaron los siguientes géneros de nemátodos: <i>Capillaria> spp., <i>Contracaecum> spp., <i>Cucullanus> spp. y <i>Raphidascaris> spp.; el último género fue el más representativo.
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Directory of Open Access Journals (Sweden)
Arias Luis Alejandro
2007-06-01
Full Text Available
El principal limitante fitosanitario del cultivo de lechuga (<i>Lactuca sativai> L. es la enfermedad conocida como moho blanco causada por el hongo <i>Sclerotinia sclerotiorumi> (Lib. de Bary. Con el fin de realizar un aporte al conocimiento de esta enfermedad y establecer protocolos para su control en campo se realizó un experimento entre los meses de febrero y mayo de 2006 en el cual se compararon tres métodos de control: aplicación de 0,5 kg· ha-1 de procimidona, dosis de conidiosporas (<i>Trichoderma harzianumi> DSM 14944 de 0,25 g· m-2 de suelo, solarización, integración de solarización con procimidona y solarización con <i>T. harzianumi>. Se evaluó la incidencia de la enfermedad, densidad de inóculo en el suelo antes y al finalizar el ensayo, temperatura del suelo durante la solarización y productividad. La densidad promedio del inóculo fue de 1,67 esclerocios de <i>S. sclerotiorumi> por100 g de suelo al inicio y 2,07 esclerocios por 100 g de suelo al finalizar el ensayo. La temperatura del suelo solarizado se incrementó hasta 50°C con un promedio de 34,69°C hacia el medio día. Los porcentajes de incidencia de la enfermedad fueron: con procimidona, 3,3%; con <i>T. harzianumi>, 10,0%; con solarización, 15,8%; con solarización y procimidona, 8,8%; con solarización y <i>T. harzianumi>, 9,2% y testigo 18,3%. No se presentaron diferencias significativas en la productividad. En conclusión el control de la enfermedad fue mejor con la aplicación de procimidona y deficiente con <i>T. harzianumi> y la solarización.
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Energy Technology Data Exchange (ETDEWEB)
Jennewein, Stephen Peter [Univ. of Florida, Gainesville, FL (United States)
2013-01-01
Here, an increasing demand for renewable energy sources has spurred interest in high-biomass crops used for energy production. Species potentially well-suited for biofuel production in the seasonally wet organic Everglades Agricultural Area (EAA) of Florida include giant reed (<i>Arundo donaxi>), elephant grass (<i>Pennisetum Purpureumi>), energycane (<i>Saccharum spp.i>), and sugarcane (<i>Saccharum spp.i>). The objectives in this study were to evaluate the role of fluctuating water tables on the morphology, physiology, and early season growth of these four genotypes. The candidate genotypes were grown in a greenhouse under three water table depths, defined by distance of the water table from the soil surface: two constant water tables (-16 cm and -40 cm) along with a flood cycle (2 weeks of flood to the soil level followed by 2 weeks at -40 cm from the soil level). The genotypes included CP 89-2143 (sugarcane), L 79-1002 (energycane), Merkeron (elephant grass), and wild type (giant reed). The experiment was repeated for plant cane, first ratoon, and successive plant cane crop cycles. Reductions in dry matter yield were observed among genotypes subjected to the -40 cm drained, periodically flooded (40F) water table relative to the -40 cm constant (40C) or -16 cm constant (16C). Plant cane dry weights were reduced by 37% in giant reed, 52% in elephant grass, 42% in energycane, and 34% in sugarcane in the 40F compared to 40C water table treatments. Similarly, in the first ratoon crop dry weights were reduced by 29% in giant reed, 42% in elephant grass, 27% in energycane, and 62% in sugarcane. In plant cane and successive plant cane, average total dry weight was greatest for elephant grass whereas ratoon total dry weight was greatest for energycane. Genotype had more pronounced effects on physiological attributes than water table including the highest stomatal conductance and SPAD values in giant reed, and the highest stalk populations in elephant grass and
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NATO Reference Mobility Model. Edition I. Users Guide. Volume I
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
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Effects of GeI2 or ZnI2 addition to perovskite CH3NH3PbI3 photovoltaic devices
Tanaka, Hiroki; Ohishi, Yuya; Oku, Takeo
2018-01-01
CH3NH3PbI3 added with GeI2 or ZnI2 perovskite photovoltaic devices were fabricated characterized. The surface coverages of the perovskite layers were improved by the addition of GeI2 or ZnI2. Formation of PbI2 observed for the pristine CH3NH3PbI3 was suppressed by the GeI2 or ZnI2 addition, which resulted in the improvement of the conversion efficiencies of the perovskite photovoltaic devices.
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Directory of Open Access Journals (Sweden)
Karol Račka
2015-05-01
Full Text Available Introduction and objective. The aims of the study were: 1 to detect antibodies against T[i]oxoplasma gondii[/i] from wild boar meat; 1 establish seroprevalence of toxoplasmosis in the wild boar population; 3 establish risk factors concerned in higher possible seroprevalence; 4 to estimate the usefulness of meat juice for detection of [i]T. gondii[/i] antibodies in wild boar. Material and methods. Diaphragm meat juice samples from 656 wild boar ([i]Sus scrofa[/i] were collected during the hunting seasons between September 2008 – October 2010 from 9 districts of the Czech Republic. The samples were stratified per age category into 2 groups: piglets (n = 279 and yearlings together with adults (n = 377. The in-house ELISA test was used for the detection of antibodies against [i]T. gondii [/i]from the meat juice samples. Results. Antibodies against [i]T. gondii [/i]were detected by in-house ELISA in 260 of 656 wild boars (40% with 26% prevalence in piglets (72/279 and 50% prevalence in yearlings and adults (188/377. The district total seroprevalences ranged between 32% – 59%, with a significantly higher prevalence in the district of Havlíčkův Brod (59%. Statistically significant differences (p-value < 0.05 were found between 2 age categories, and between 9 districts, with a significant variability in the district of Havlíčkův Brod. Seroprevalence correlated positively with farm density, but without any statistical significance. Conclusion. The obtained results indicate that consumption of raw or undercooked meat from wild boars can carry an important risk of toxoplasma infection. Post mortem detection of antibodies in meat juice samples using ELISA is a useful alternative to blood serum examination. In addition, a diaphragm sample has been well-proven as a matrix sample for the contemporaneous diagnostics of trichinellosis and toxoplasmosis.
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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...
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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,...
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Sårbare børn og unge i tandplejeregi i Danmark
DEFF Research Database (Denmark)
Uldum, Birgitte; Christensen, Hanne Nødgaard; Haubek, Dorte
2016-01-01
Hvorledes identificeres sårbare børn og unge i tandplejeregi i Danmark? I denne artikel sættes fokus på danske sårbare børn og unge i tandplejeregi og på udviklingen I Danmark siden 2009. En dansk spørgeskemaundersøgelse om tandplejens erfaring fra 2008 omtales kort. Der lægges vægt på det odonto...
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Production of gaseous radiotracers CH{sub 3}I and I{sub 2} through Na{sup 123}I salt
Energy Technology Data Exchange (ETDEWEB)
Candeiro, R.E.M., E-mail: ricardocandeiro@cnen.gov.b [Comissao Nacional de Energia Nuclear (DIFOR/CNEN-CE), Fortaleza, CE (Brazil). Distrito de Fortaleza; Brandao, L.B. [Instituto de Engenharia Nuclear (IEN/CNEN-RJ), Rio de Janeiro, RJ (Brazil); Pereira, W.P. [Universidade Federal Fluminense (UFF), Niteroi, RJ (Brazil)
2011-07-01
The objective of the present work was to develop, separately, methodology for production of two gaseous tracers through the sodium iodide NaI marked with {sup 123}I. Found in the nature in form different, the iodine has been used in diverse works in the area of the industry and health. These two forms of the gaseous iodine, the methyl iodide, CH{sub 3}I, and molecular iodine, I{sub 2}, are very unstable and volatile in the ambient temperature and presents different problems in clean-up and monitoring systems. The syntheses were processed with sodium iodide (NaI) 1M aqueous solution marked with 1{sup 23I}. The production of gas I{sub 2} was realized with in chlorine acid (HCl) and sodium iodate salt (NaIO{sub 3}) and the CH{sub 3}I was used, the salt of NaI and the reagent (CH{sub 3}){sub 2}SO{sub 4}. The production of gases was initially realized through in unit in glass with an inert material and the purpose was to study the kinetic of reaction and to determine the efficiency of production. The two synthesis occurs in the reaction bottle and after of produced, the gas is stored in the collect bottle that contains a starch solution for fixed the I{sub 2}, and in syntheses of CH{sub 3}I contains a silver nitrate solution for your fixation. To determine the efficiency of production of gases, analytic tests were realized, where the consumption of iodide ions of the bottle of reaction are measured. The optimization of production of the each gaseous tracer was studied varying parameter as: concentration of iodide, concentration of acid and temperature. After, the syntheses of the radiotracers were realized in the compact unit, having been used as main reagent the salt radiated of sodium iodide, Na{sup 123}I. The transportation of elementary iodine and methyl iodine was studied by a scintillation detector NaI (2 x 2)' positioned in the reaction bottle. (author)
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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 ...
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Neutron activation analysis of 127I and 129I environmental samples
International Nuclear Information System (INIS)
Takeishi, Minoru; Namiki, Atsushi; Ishida, Junichiro; Iwai, Makoto
1987-01-01
The analytical method of 127 I and 129 I in environmental samples has been studied and the background levels of these nuclides in soils, seaweeds and milk were measured. The analytical method consists of the combustion technique and neutron activation analysis (N.A.A.). The iodine was separated from samples by ignition at about 1,000 deg C in a quartz combustion apparatus with an oxygen and a nitrogen stream, and was absorbed by small amounts of active charcoal. The iodine was then purified through the carbontetrachloride extraction method and the PdI 2 decomposition method. Irradiation was conducted by JRR-4 (n th : 8 x 10 13 n/cm 2 · sec) for 40 min. After irradiation, the iodine was purified by the solvent extraction method same as pre-irradiation extraction. Each activity of 126 I and 130 I, which was produced by 127 I(n,2n) 126 I and 129 I(n,γ) 130 I reaction respectively, was measured by γ-spectrometry using a Ge-detector. The lower detection limits of 129 I by this method were 4 x 10 -7 Bq/g for dry soil, 7 x 10 -8 Bq/g for fresh seaweeds and 7 x 10 -6 Bq/l for fresh milk, respectively. The relative standard deviation of 129 I measurements in soil and milk were less than 10 %. (author)
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Developing C# Apps for iPhone and iPad using MonoTouch
Costanich, Bryan
2011-01-01
Developing C# Applications for iPhone and iPad using MonoTouch shows you how to use your existing C# skills to write apps for the iPhone and iPad. Fortunately, there's MonoTouch, Novell's .NET library that allows C# developers to write C# code that executes in iOS. Furthermore, MonoTouch allows you to address all the unique functions of the iPhone, iPod Touch, and iPad. And the big plus: You needn't learn any Objective-C to master MonoTouch!. Former Microsoft engineer and published app-store developer Bryan Costanich shows you how to use the tools you already know to create native apps in iOS
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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.)
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Directory of Open Access Journals (Sweden)
Tello Paola
2007-12-01
Full Text Available
La mosca blanca de los invernaderos, <i>Trialeurodes vaporariorumi> (Westwood (Homoptera: Aleyrodidae es una de las plagas más importantes de cultivos de tomate bajo invernadero en la Sabana de Bogotá. Para su control tradicionalmente se acude a la aplicación de insecticidas. Sin embargo, también son contempladas liberaciones del parasitoide <i>Encarsia formosai> (Gahan (Hymenoptera: Aphelinidae. Para estandarizar el proceso de cría masiva de <i>E. formosai>, se evaluaron tres densidades diferentes de liberación de las avispas (6, 12 y 18 avispas/planta en tres frecuencias de liberación (1, 2 y 3 veces/semana, sobre plantas de tomate de ocho semanas de edad con tres niveles de infestación (15, 30 y 45 ninfas de mosca blanca/hoja, susceptibles de ser parasitadas. El mayor porcentaje de parasitismo (70% en ninfas de tercer instar se logró cuando se liberaron seis avispas por planta, dosificadas en tres momentos diferentes en la misma semana. El menor porcentaje de parasitismo (50%, se obtuvo cuando se realizó una sola liberación de 6, 12 o 18 avispas. Los resultados anteriores permiten concluir que se puede lograr un porcentaje de parasitismo en torno a un 70% de la infestación de mosca blanca (hasta 45 ninfas/hoja en plantas de tomate con pocas avispas de <i>E. formosai> (seis cuando se liberan en tres momentos diferentes.
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Differential binding of 125I-IGF-I preparations to human fibroblast monolayers
International Nuclear Information System (INIS)
Conover, C.A.; Misra, P.; Hintz, R.L.; Rosenfeld, R.G.
1988-01-01
Specific, high affinity binding of 125 I-IGF-I to the type IIGF receptor on human fibroblast monolyaers was not altered by varying feeding schedules, serum lots, washing procedures, or incubation times and temperatures. However, markedly different competitive binding curves were obtained when different iodinated IGF-I preparations were used. Five of six radioligands bound preferentially to the type IIGF receptor on human fibroblast monolayers, with 50% displacement at 4-8 μg/l unlabelled IGF-I; with one radioligand a paradoxical 20-200% increase in 125 I-IGF-I binding was observed at low concentrations of unlabelled IGF-I, while concentrations as high as 100 μg/l IGF-I failed to displace this radioligand. The latter binding pattern cannot be accounted for by 125 -I-IGF-I binding to the type II IGF receptor. These data indicate that various radioligands may have preferential affinities for different IGF-I binding sites on human fibroblast monolayers. (author)
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Wolfenden, C; Wittkowski, A; Hare, D J
2017-11-01
The prevalence of autism spectrum disorder (ASD) in many genetic disorders is well documented but not as yet in Mucopolysaccharidosis type III (MPS III). MPS III is a recessively inherited metabolic disorder and evidence suggests that symptoms of ASD present in MPS III. This systematic review examined the extant literature on the symptoms of ASD in MPS III and quality assessed a total of 16 studies. Results indicated that difficulties within speech, language and communication consistent with ASD were present in MPS III, whilst repetitive and restricted behaviours and interests were less widely reported. The presence of ASD-like symptoms can result in late diagnosis or misdiagnosis of MPS III and prevent opportunities for genetic counselling and the provision of treatments.
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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...
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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.
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DEFF Research Database (Denmark)
Jørgensen, Louise von Gersdorff; Kania, Per Walter; Rasmussen, Karina Juhl
2017-01-01
produced in E. coli and one in insect cells) were combined and injected i.p., and subsequently, protection and antibody responses were analysed. Both the experimental and the benchmark vaccine induced partial but significant protection against I. multifiliis when compared to control fish. Specific antibody...
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Impact of high (131)I-activities on quantitative (124)I-PET
DEFF Research Database (Denmark)
Braad, P E N; Hansen, Søren B.; Høilund-Carlsen, P F
2015-01-01
relevant [Formula: see text]I/[Formula: see text]I-activities were performed on a clinical PET/CT-system. Noise equivalent count rate (NECR) curves and quantitation accuracy were determined from repeated scans performed over several weeks on a decaying NEMA NU-2 1994 cylinder phantom initially filled...... [Formula: see text]I-activities was good and image quantification unaffected except at very high count rates. Quantitation accuracy and contrast recovery were uninfluenced at [Formula: see text]I-activities below 1000 MBq, whereas image noise was slightly increased. The NECR peaked at 550 MBq of [Formula......: see text]I, where it was 2.8 times lower than without [Formula: see text]I in the phantom. Quantitative peri-therapeutic [Formula: see text]I-PET is feasible....
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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...
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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....
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The thermodynamic characteristics of vaporization in the NaI-PrI3 system
Motalov, V. B.; Kudin, L. S.; Markus, T.
2009-05-01
The vaporization of the NaI-PrI3 quasi-binary system was studied by high-temperature mass spectrometry over the whole concentration range. At 623-994 K, saturated vapor contained not only (NaI) n and (PrI3) n molecules ( n = 1, 2) and Na+(NaI) n ( n = 0-4) and I-(PrI3) n ( n = 1-2) ions but also mixed molecular and ionic associates recorded for the first time (NaPrI4, Na2PrI5, NaPrI{3/+}, Na2PrI{4/+}, Na3PrI{5/+}, Na4PrI{6/+}, NaPrI{5/-}, and NaPr2I{8/-}). The partial vapor pressures of molecules were calculated, and the equilibrium constants of the dissociation of neutral and charged associates were measured. The enthalpies of molecular and ion-molecular reactions were determined, and the enthalpies of formation of gaseous molecules and ions were obtained.
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Exploring the iPhone Backup made by iTunes
Directory of Open Access Journals (Sweden)
Mario Piccinelli
2011-09-01
Full Text Available The iPhone mobile from Apple Inc. is one of the most notable phones on the market thanks to its simple and user-friendly interface and ever growing pool of available high quality applications for both personal and business use. The increasing use of iPhone mobiles leads forensics practitioners towards the need for tools to access and analyze the information stored in the device. This research aims at describing how to forensically analyze a logical backup of an iPhone made by the Apple iTunes utility, understanding its structure and creating a simple tool to automate the process of decoding and analyzing the data. It was found that significant data of forensic value such as e-mail messages, text and multimedia messages, calendar events, browsing history, GPRS locations, contacts, call history and voicemail recordings can be retrieved using this method of iPhone acquisition.
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Directory of Open Access Journals (Sweden)
Flórez Víctor Julio
2008-08-01
Full Text Available
En <i>Solidago> × <i>luteus> (M.L. Greene Brouillet y Semple (= × <i>Solidaster hybridusi>,× <i>S. luteusi>, planta comercializada principalmente para flor de corte, el proceso de inducción floral es dependiente de las condiciones fotoperiódicas ambientales. Con el objetivo de determinar la acción de las citoquininas en este proceso, se analizó su presencia en condiciones fotoperiódicas opuestas: días largos (DL de 18 h y días cortos (DC de 8 h. En la primera fase del ensayo, con el fin de analizar la respuesta de las plantas a fitohormonas con frecuencia semanal de aplicación, se realizaron los siguientes tratamientos: 1 ácido giberélico (GA3 10-4M, 6-benzilaminopurina (6-BA 4x10-4 M y GA3 10-4 M más 6-BA 4x10-4 M; 2 GA3 10-4M, kinetina (KI 4x10-4 M y GA3 10-4 M más KI 4x10-4 M y 3 KI 4x10-4 M. Con el objeto de conocer la concentración endógena de hormonas, en la segunda fase los extractos vegetales se analizaron a través de cromatografía en capa fina y cuantificados por el ensayo inmunoenzimático de Elisa, previa separación por cromatografía líquida de alta eficiencia. Los resultados permitieron determinar la actividad citoquinínica en extractos provenientes de hojas y de botones florales de plantas en el inicio de los tratamientos, en DL y en DC. Se demostró que la aplicación de KI acelera la antesis floral, evidenciando la relación de citoquininas endógenas con el proceso de desarrollo floral. Por último, de los resultados obtenidos de los extractos sometidos a Elisa se sugiere la participación de las citoquininas en el proceso de antesis floral de <i>S.>× <i>luteus>, donde la iPA (isopentenil adenosina tendría una acción importante al ser transportada rápidamente de la hoja al botón floral en DC, posibilitando tal vez la mayor
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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....
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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...
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<i>PALB2i>, <i>CHEK2i> and <i>ATM i>rare variants and cancer risk
DEFF Research Database (Denmark)
Southey, Melissa C; Goldgar, David E; Winqvist, Robert
2016-01-01
2 mutations. We aimed to estimate the relative risks associated with specific rare variants in PALB2, CHEK2 and ATM via a multicentre case-control study. METHODS: We genotyped 10 rare mutations using the custom iCOGS array: PALB2 c.1592delT, c.2816T>G and c.3113G>A, CHEK2 c.349A>G, c.538C>T, c.715G...
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<i>Skjaldar þáttr Danakonungsi>
DEFF Research Database (Denmark)
Lavender, Philip Thomas
2016-01-01
In this article I discuss a reference in Illuga saga Gríðarfóstra to a Skjaldar þáttur ok Hermanns. Previous commentators have said that this reference is either made up or alludes to a now lost saga. I show that the reference to the saga is not a fabrication: the narrative of Skjöldr and Hermann...
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Human conditions of insulin-like growth factor-I (IGF-I) deficiency
2012-01-01
Insulin-like growth factor I (IGF-I) is a polypeptide hormone produced mainly by the liver in response to the endocrine GH stimulus, but it is also secreted by multiple tissues for autocrine/paracrine purposes. IGF-I is partly responsible for systemic GH activities although it possesses a wide number of own properties (anabolic, antioxidant, anti-inflammatory and cytoprotective actions). IGF-I is a closely regulated hormone. Consequently, its logical therapeutical applications seems to be limited to restore physiological circulating levels in order to recover the clinical consequences of IGF-I deficiency, conditions where, despite continuous discrepancies, IGF-I treatment has never been related to oncogenesis. Currently the best characterized conditions of IGF-I deficiency are Laron Syndrome, in children; liver cirrhosis, in adults; aging including age-related-cardiovascular and neurological diseases; and more recently, intrauterine growth restriction. The aim of this review is to summarize the increasing list of roles of IGF-I, both in physiological and pathological conditions, underlying that its potential therapeutical options seem to be limited to those proven states of local or systemic IGF-I deficiency as a replacement treatment, rather than increasing its level upper the normal range. PMID:23148873
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Human conditions of insulin-like growth factor-I (IGF-I deficiency
Directory of Open Access Journals (Sweden)
Puche Juan E
2012-11-01
Full Text Available Abstract Insulin-like growth factor I (IGF-I is a polypeptide hormone produced mainly by the liver in response to the endocrine GH stimulus, but it is also secreted by multiple tissues for autocrine/paracrine purposes. IGF-I is partly responsible for systemic GH activities although it possesses a wide number of own properties (anabolic, antioxidant, anti-inflammatory and cytoprotective actions. IGF-I is a closely regulated hormone. Consequently, its logical therapeutical applications seems to be limited to restore physiological circulating levels in order to recover the clinical consequences of IGF-I deficiency, conditions where, despite continuous discrepancies, IGF-I treatment has never been related to oncogenesis. Currently the best characterized conditions of IGF-I deficiency are Laron Syndrome, in children; liver cirrhosis, in adults; aging including age-related-cardiovascular and neurological diseases; and more recently, intrauterine growth restriction. The aim of this review is to summarize the increasing list of roles of IGF-I, both in physiological and pathological conditions, underlying that its potential therapeutical options seem to be limited to those proven states of local or systemic IGF-I deficiency as a replacement treatment, rather than increasing its level upper the normal range.
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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...
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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...
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High proportion of mannosidosis and fucosidosis among lysosomal storage diseases in Cuba.
Menéndez-Sainz, C; González-Quevedo, A; González-García, S; Peña-Sánchez, M; Giugliani, R
2012-08-13
Although lysosomal storage disorders (LSDs) are considered individually rare, as a group they present a non-negligible frequency. Few studies have been made of populational occurrence of LSDs; they have been conducted predominantly on Caucasian populations. We studied the occurrence of LSDs in Cuba. Data from individuals who had been referred to the Institute of Neurology and Neurosurgery in Havana from hospitals all over the country between January 1990 and December 2005 were analyzed. This institute was the only laboratory to provide enzyme-based diagnostic testing for 19 LSDs in Cuba during this period. Occurrence rates were calculated by dividing the number of postnatal diagnoses by the number of births during the study period. The combined occurrence of LSDs in Cuba was 5.6 per 100,000, lower than that reported in other studies conducted on Caucasian populations. The most frequent individual LSDs were: mucopolysaccharidosis type I (1.01 per 100,000) and, surprisingly, alpha-mannosidosis (0.72 per 100,000) and fucosidosis (0.62 per 100,000). These findings may be related to specific genetic characteristics and admixture of the Cuban population. This is the first comprehensive study of the occurrence of LSDs in Cuba. We conclude that the epidemiology of these diseases can vary regionally, and we stress the need for similar surveys in other Latin American countries.
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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
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Pajamų iš tiesioginių mokesčių tyrimas Lietuvoje
Stelmakovienė, Reda
2012-01-01
Bakalauro baigiamąjame darbe atskleidžiama tiesioginių mokesčių vieta Lietuvos mokesčių sistemoje, išskiriant mokesčių rūšis priskiriamas prie tiesioginių mokesčių grupės, nagrinėjami GPM, pelno mokesčio ir turto mokesčių (nekilnojamojo turto mokesčio, žemės mokesčio, paveldimo turto mokesčio) apmokestinimo instrumentarijai. Nagrinėjama nacionalinio biudžeto mokestinių pajamų struktūrą, dinamika 2006-2010 m., atskleidžiama pajamų iš tiesioginių mokesčių lyginamieji svoriai nacionalinio biudže...
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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....
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Razvoj i primena hemometrijskih metoda za klasifikaciju i procenu kvaliteta vode
Živojinović, Dragana Z.
2013-01-01
Savremeno društvo karakteriše intenzivna industrijalizacija i urbanizacija, što ima za posledicu sve obimnije iscrpljivanje prirodnih resursa i sve opasnije ugrožavanje životne sredine. U uslovima globalnog razvoja, briga o vodi predstavlja pitanje opstanka civilizacije. Stoga upravljanje vodama i kontrola kvaliteta voda postaju suštinska društvena potreba. Timovi stručnjaka iz različitih oblasti pokušavaju da nađu obrasce i modele za modelovanje parametara kvaliteta vode i pre...
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<i>Orobanche olbiensisi> (Coss. Nyman, taxon minusvalorado del Mediterráneo
Directory of Open Access Journals (Sweden)
Pujadas Salvà, Antonio J.
2000-12-01
Full Text Available <i>Orobanche olbiensisi> is reported for the first time in the Iberian flora. growing on coastal sand-dune ecosystems of Alicante province (southeastern Spain. This taxon was neglected by most of European authors. probably due to its affinity to <i>O.mutelii>, with which it has been usually misidentified. A detailed description and drawing of the Alicantine plants are presented, and data on their ecology, phytosociology and distribution are also reported. Main differencess with <i>O. muteliii> are pointed out in an identification key.
Se indica, por primera vez para la flora ibérica. la presencia de <i>Orobanche olbiensisi>, a partir de poblaciones localizadas en ecosistemas dunares costeros de la provincia de Alicante. Se trata de un taxon que ha pasado desapercibido casi desde su descripción, probablemente por su afinidad con <i>O. muteliii>, planta con la que ha sido confundida. Se aporta una descripción e iconografía detalladas del material alicantino, así como datos sobre su ecología. fitosociología y distribución. Se destacan las diferencias más significativas con <i>O.mutelii> en una clave de identificación. -
International Nuclear Information System (INIS)
Zhang, Daopeng; Zhao, Zengdian; Wang, Ping; Chen, Xia
2012-01-01
Two pyridinecarboxamide dicyanidecobalt(III) building blocks and two mononuclear seven-coordinated macrocycle manganese(II) compounds have been rationally selected to assemble cyanide-bridged heterobimetallic complexes, resulting in three cyanide-bridged Co"I"I"I-Mn"I"I complexes. Single X-ray diffraction analysis show that these complexes {[Mn(L"1)][Co(bpb)]}ClO_4·CH_3OH·0.5H_2O (1), {[Mn(L"2)][Co(bpb)]}ClO_4·0.5CH_3OH (2) and {[Mn(L"1)][Cobpmb]}ClO_4·H_2O (3) (L"1 = 3,6-diazaoctane-1,8-diamine, L"2 = 3,6-dioxaoctano-1,8- diamine: bpb"2"- = 1,2-bis(pyridine-2-carboxamido)benzenate, bpmb"2"- = 1,2-bis(pyridine-2-carboxamido)-4- methyl-benzenate) all present predictable one-dimensional single chain structures. The molecular structures of these one-dimensional complexes consists of alternating units of [Mn(L)]"2"+ (L = L"1 or L"2) and [Co(L')(CN)_2]"- (L' = bpb"2"-, or bpmb"2"-), forming a cyanide-bridged cationic polymeric chain with free ClO_4"- as the balance anion. The coordination geometry of manganese(II) ion in the three one-dimensional complexes is a slightly distorted pentagonal-bipyrimidal with two cyanide nitrogen atoms at the trans positions and N_5 or N_3O_2 coordinating mode at the equatorial plane from ligand L"1 or L"2. Investigation over magnetic properties of these complexes reveals that the very weak magnetic coupling between neighboring Mn(II) ions connected by the diamagnetic dicyanidecobalt(III) building block. A best-fit to the magnetic susceptibility of complex 1 leads to the magnetic coupling constants J = .0.084(3) cm"-"1
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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...
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Comparison of four HTLV-I and HTLV-I + II ELISAs
Vrielink, H.; Reesink, H.; Habibuw, M.; Schuller, M.; van der Meer, C.; Lelie, P.
1999-01-01
BACKGROUND: Various countries require blood donor screening using assays applying specific HTLV-I and HTLV-II antigens. We evaluated the sensitivity and specificity of 4 anti-HTLV-I + II ELISAs (Abbott, Murex, Organon Teknika and Ortho). METHODS: Panel A consisted of HTLV-I-positive individuals (n =
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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...
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International Nuclear Information System (INIS)
Thibaut L, Julio; Ditzel, G.; Vargas, L; Born, R; Deppe G, Rodolfo
1996-01-01
Two urographic methods were compared: the intravenous urographic bolus (i.u.b.) and the intravenous urographic infusion (i.u.i.). In both methods, two groups of seven healthy adult dogs of both sexes, weighing7.0 to 16.5 kg were used and were anaesthesized with 2% thiopentone sodium in doses of 20 mg/kg via cephalica. In the i.u.b., meglumine diatrizoate (Hypaque-M, 60%) was injected via saphena with a concentration of 282 mg of iodine per mi in doses of 564 mg of iodine per kg. In the i.u.i., meglumine diatrizoate was injected via saphena by drip infusion with a concentration of 200 mg of iodine per mi in doses of 500 mg of iodine per kg. Three series of two X-rays each were taken in ventrodorsal projection 1, 4 and 8 min and left lateral recumbency 30 sec after administering the contrast medium. The X-ray plates obtained were analyzed and compared intra and inter group considering the advance speed of the contrast medium, the radiographic density and outline, and kidney size. The advance speed of the contrast medium was higher in the i.u.i., reaching the kidney, ureter and bladder 1 min after administration in both projections; in ventrodorsal projections in the i.u.b. only the kidneys were reached while in the left lateral recumbency, the kidney and ureters were reached [es
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D-Allose catabolism of <i>Escherichia colii>
DEFF Research Database (Denmark)
Poulsen, Tim S.; Chang, Ying-Ying; Hove-Jensen, Bjarne
1999-01-01
Genes involved in allose utilization of Escherichia coli K-12 are organized in at least two operons, alsRBACE and alsI, located next to each other on the chromosome but divergently transcribed. Mutants defective in alsI (allose 6-phosphate isomerase gene) and alsE (allulose 6-phosphate epimerase...... gene) were Als-. Transcription of the two allose operons, measured as β-galactosidase activity specified by alsI-lacZ+ or alsE-lacZ+ operon fusions, was induced by allose. Ribose also caused derepression of expression of the regulon under conditions in which ribose phosphate catabolism was impaired....
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Kontinuitet i diskontinuitet naseljenosti Istre na razmeđu prapovijest i antike
Bulić, Davor
2014-01-01
Naseljavanje Italika na Istarski poluotok krajem republikanskog i početkom carskog razdoblja uvjetovalo je u znatnoj mjeri i promjene mjesta naseljavanja, kao i vrste naselja. Ove je promjene moguće sagledati u procesu napuštanja gradinskih naselja i naseljavanja lokacija u nizinskim predjelima u okviru rimskih ruralnih stambeno-gospodarskih kompleksa (villa rustica). Iako je napuštanje predrimskih naselja na području kolonijskih agera očit i prevladavajući oblik promjene mjesta naseljavanja,...
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Virtual reality, augmented reality…I call it i-Reality.
Grossmann, Rafael J
2015-01-01
The new term improved reality (i-Reality) is suggested to include virtual reality (VR) and augmented reality (AR). It refers to a real world that includes improved, enhanced and digitally created features that would offer an advantage on a particular occasion (i.e., a medical act). I-Reality may help us bridge the gap between the high demand for medical providers and the low supply of them by improving the interaction between providers and patients.
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Synthesis of (125) I-lamivudine and (125) I-lamivudine-ursodeoxycholic acid codrug.
Motaleb, M A; Abo-Kul, M; Ibrahim, Samy M; Saad, Shokry M; Arafat, Muhammad
2016-09-01
The preparation of (125) I-lamivudine ((125) I-3TC) and (125) I-lamivudine-ursodeoxycholic acid codrug ((125) I-3TC-UDCA), suitable for comparative biodistribution studies, is described. The synthesis of the unlabeled precursor 3TC-UDCA proceeds in an 11.6% yield, and the radiolabelling yields for (125) I-3TC and (125) I-3TC-UDCA were 89 and 92%, respectively. The final products are radiochemically pure (greater than 98%). Copyright © 2016 John Wiley & Sons, Ltd.
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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
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Specialiseringsmodul i Læreruddannelsen - inklusion i skolen og social innovation
DEFF Research Database (Denmark)
Gottlieb, Lotte; Johannesen, Rikke
2017-01-01
Modulet "Inklusion i skolen og socialt entreprenørskab" retter sig mod det store overlap mellem inklusion og social innovation/socialt entreprenørskab. Innovation og entreprenørskab indgår derfor i modulet som et didaktisk greb både indholds- og formmæssigt.......Modulet "Inklusion i skolen og socialt entreprenørskab" retter sig mod det store overlap mellem inklusion og social innovation/socialt entreprenørskab. Innovation og entreprenørskab indgår derfor i modulet som et didaktisk greb både indholds- og formmæssigt....
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Veiledning i praksis. Om praksisopplæring i norsk allmennlærerutdannelse
Directory of Open Access Journals (Sweden)
Kaare Skagen
2009-05-01
Full Text Available Artikkelen presenterer to nyere studier av veiledning i praksisopplæring i allmennlærerutdannelsen. Hovedfunnene drøftes i forhold til noen tidligere nordiske forskningsstudier. Studiene redegjør for intensjoner og realiteter i praksisopplæringen med fokus på mål, innhold og form i praksisveiledningen. Materialet er ramme- og fagplaner og intervjuer og observasjoner ved tre praksisskoler. Veiledningen drøftes i forhold til teori om veiledning. De to studiene som presenteres, konkluderer med at avstanden mellom teoridel og praksisdel i lærerutdannelsen fortsatt eksisterer. Det blir konstatert både fellestrekk og forskjeller mellom skolene når det gjelder graden av individuell veiledning og gruppeveiledning og individuell studentundervisning. Innholdsfokus i veiledningen varierte. Veilederrollen varierte mellom ettergivenhet og pågåenhet. Noen forskningsstudier hevder at øvingslærere praktiserer teknologisk veiledning. Artikkelen argumenterer for at innvielse i undervisningstradisjonen er nødvendig.
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30 CFR 57.22202 - Main fans (I-A, I-B, I-C, II-A, III, V-A, and V-B mines).
2010-07-01
... 30 Mineral Resources 1 2010-07-01 2010-07-01 false Main fans (I-A, I-B, I-C, II-A, III, V-A, and V... Main fans (I-A, I-B, I-C, II-A, III, V-A, and V-B mines). (a) Main fans shall be— (1) Installed on the... mines, provided with an automatic signal device to give an alarm when the fan stops. The signal device...
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iOS Sensor Apps with Arduino Wiring the iPhone and iPad into the Internet of Things
Allan, Alasdair
2011-01-01
Turn your iPhone or iPad into the hub of a distributed sensor network with the help of an Arduino microcontroller. With this concise guide, you'll learn how to connect an external sensor to an iOS device and have them talk to each other through Arduino. You'll also build an iOS application that will parse the sensor values it receives and plot the resulting measurements, all in real-time. iOS processes data from its own onboard sensors, and now you can extend its reach with this simple, low-cost project. If you're an Objective-C programmer who likes to experiment, this book explains the basi
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Bog i besmrtnost – drugo i treće najvažnije pitanje
Djurić, Marko P.
2013-01-01
Autor u ovom članku sagledava pitanje o Bogu i pitanje o čovjekovoj besmrtnosti kao čovjekovo drugo i treće najvažnije pitanje. Pitanje o čovjekovoj sreći smatra prvim i najvažnijim. Prema njegovu shvaćanju sva tri pitanja međusobno se prožimlju, ne isključujući se. Bez vjere u objavljenog Boga i vjerovanja u vlastitu besmrtnost čovjek neće moći doživjeti najdublju i najpotpuniju sreću. Stoga se ovo izlaganje isključivo tiče eshatološkog vremena i budućnosti. Posljedica č...
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Flujo de control en iOS Flow control in iOS
Directory of Open Access Journals (Sweden)
Franklin Hernández Castro
2012-11-01
Full Text Available El objetivo de este artículo es explicar los flujos de control que se usan en la programación de las aplicaciones en iOS, con el fin de resumir los aspectos más relevantes que se deben tomar en cuenta para programar una tarea a ser realizada por un dispositivo móvil del tipo iPhone o iPad. Debido a que el ambiente iOS es estrictamente orientado a objetos (OOP, los flujos de control no son obvios; además, los estándares de la firma Apple® definen patrones de diseño en el sistema que son altamente recomendados en este tipo de diseño. En este artículo se introducen algunos de ellos.This paper explain the control flows that are used by programming applications in iOS, trying to summarize the most important aspects to be considered by programming mobile devices like iPhone and iPad. Because iOS environment, is strictly a object-oriented one(OOP, control flows are not obvious, besides Apple® use design patterns highly recommended in this type of programming. Here we introduces some of them.
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Temporalizar la historia. Sobre la <i>Historik> de Reinhart Koselleck
Directory of Open Access Journals (Sweden)
Chignola, Sandro
2007-12-01
Full Text Available Reinhart Koselleck’s <i>Historik>, following german classic hermeneutics, represents an important philosophical attempt in formalizing the trascendental categories for historical experience of time. The article focuses on Koselleck’s attemp and try to demonstrate that, referring to Kant’s philosophy of history and as Michel Foucault did, Koselleck assumes, in a circular way, the formal criteria for his <i>Historik> from the experience of temporalization and acceleration of history which mark modernity as an epoch.
La <i>Historik> de Reinhart Koselleck representa un importante intento filosófico —llevado a cabo siguiendo la estela de la hermenéutica clásica alemana— de formalizar las categorías trascendentales de la experiencia histórica del tiempo. El ensayo pone a prueba la hipótesis de Koselleck y pretende demostrar cómo éste, refiriéndose a Kant y de manera análoga a Foucault, extrae circularmente los criterios formales para su <i>Historik> a partir de la experiencia de aceleración y temporalización de la historia que caracteriza a la modernidad como época. -
Directory of Open Access Journals (Sweden)
Niels Hannibal
2005-02-01
Full Text Available Denne artikel indeholder præsentation og analyse af data indsamlet på 5 psykiatriske institutioner i Danmark, der har musikterapi i behandlingen. Formålet med undersøgelsen er dels at beskrive den patientgruppe der modtager musikterapi i psykiatrien, og dels undersøge om enkelte variable kan prædiktere drop-out fra behandlingen. Både demografiske, psykiatriske og terapeutiske data indsamles fra 91 patienter, der har modtaget musikterapi i perioden august 2003 til juli 2004. Undersøgelsen viser, at i denne gruppe rekrutteres patienterne overvejende fra hospitalerne, de har ofte enten skizofreni lidelser eller personlighedsforstyrrelser. 16 % dropper i gennemsnit ud af behandlingen. Der var ingen enkelte variable, der prædikterede drop-out fra behandlingen.
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Fra aber i anekdoter til katte i kasser
DEFF Research Database (Denmark)
Gjerløff, Anne Katrine
2009-01-01
Artiklen beskriver hvordan Charles Darwin og hans dicipel georges Romanes i deres arbejde med dyrenes åndsevner i 1870'erne og 1880'erne søgte at vise, hvor tæt beslægtede de klogeste dyr, såsom aber, hunde og heste, var med mennesket og dermed underbyggede den evolutionære forbindelse. Darwin og...... Romanes baserede i høj grad deres konklusioner på anekdotisk kildemateriale, og blev omkring 1900 stærkt kritiseret af den behavioristiske skole inden for psykologien, bl.a. af Edward Thorndike der forsøgte at at afprøve teorierne eksperimentelt. Udgivelsesdato: 12.2.09...
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Association of Taq I, Fok I and Apa I polymorphisms in Vitamin D Receptor (VDR) gene with leprosy.
Neela, Venkata Sanjeev Kumar; Suryadevara, Naveen Chandra; Shinde, Vidya Gouri; Pydi, Satya Sudheer; Jain, Suman; Jonnalagada, Subbanna; Singh, Surya Satyanarayana; Valluri, Vijaya Lakshmi; Anandaraj, M P J S
2015-06-01
Vitamin D Receptor (VDR) is a transacting transcription factor which mediates immunomodulatory function and plays a key role in innate and adaptive immune responses through its ligand and polymorphisms in VDR gene may affect its regulatory function. To investigate the association of three VDR gene polymorphisms (TaqI rs731236, FokI rs2228570 and ApaI rs7975232) with leprosy. The study group includes 404 participants of which 222 were leprosy patients (paucibacillary=87, multibacillary=135) and 182 healthy controls. Genotyping was done using PCR-RFLP technique. Statistical analysis was performed using SNP Stats and PLINK software. The VDR FokI (rs2228570) ff genotype, ApaI (rs7975232) AA, Aa genotype and haplotype T-f-a, T-F-A were positively associated with leprosy when compared to healthy controls. The two variants at Fok and Apa positions in VDR gene are significantly associated with leprosy. Genotypes at FokI (ff), ApaI (aa) and haplotype (T-F-a, T-f-a) may contribute to the risk of developing leprosy by altering VDR phenotype/levels subsequently modulation of immune response. Copyright © 2015 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.
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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...
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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....
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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...
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International Nuclear Information System (INIS)
Odin, I.N.; Grin'ko, V.V.; Kozlovskij, V.F.; Safronov, E.V.
2005-01-01
Mutual system PbSe + Ag 2 I 2 = Ag 2 Se + PbI 2 is investigated. It is shown that diagonal Ag 2 Se-PbI 2 is stable. Liquidus surface and isothermal section at 633 K of phase diagram of PbSe-Ag 2 Se-PbI 2 system are built. Transformations directing to crystallization metastable ternary compound forming in PbSe-PbI 2 system and metastable polytype modifications of lead iodide in PbSe-Ag 2 Se-PbI 2 system at 620-685 K are studied. By hardening from molten state (1150-1220 K) new interstitial metastable phases crystallizing in CdCl 2 structural type are obtained in PbSe-Ag 2 Se-PbI 2 and PbSe + CdI 2 = CdSe + PbI 2 systems [ru
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Combinatorial Reductions for the Stanley Depth of I and S/I
Energy Technology Data Exchange (ETDEWEB)
Keller, Mitchel T.; Young, Stephen J.
2017-09-07
We develop combinatorial tools to study the realtionship between the Stanley depth of a monomial ideal I and the Stanley depth of its compliment S/I. Using these results we prove that if S is a polynomial ring with at most 5 indeterminates and I is a square-free monomial ideal, then the Stanley depth of I is strictly larger than the Stanley depth of S/I. Using a computer search, we extend the strict inequality to the case of polynomial rings with at most 7 indeterminates. This partially answers questinos asked by Proescu and Qureshi as well as Herzog.
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Iodine (I-129 and I-127) in aerosols from northern Europe
DEFF Research Database (Denmark)
Englund, E.; Aldahan, A.; Hou, Xiaolin
2010-01-01
Huge amount of 129I has been emitted to the environment during the last decades from a variety of anthropogenic nuclear activities. Aerosols represent a carrier of iodine in the atmosphere in addition to gaseous forms for which data are lacking in Sweden. In this study the first time series...... of iodine isotopes (129I and 127I) in aerosols covering the period of 1983 to 2008 from two sites in Sweden are presented. The results show that both 129I and 127I concentrations are considerably higher in southern compared to northern Sweden. Furthermore there is a significant difference in the relative...
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Hvordan anvender studerende forhandling i computerkonferencer i forbindelse med deres læring?
Directory of Open Access Journals (Sweden)
Pernille Rattleff
2006-05-01
Full Text Available Første gang publiceret i UNEV nr. 9: Evaluering og feedback i netstøttet uddannelse, sept. 2006, red. Simon Heilesen.
ISSN 1603-5518.
I denne artikel fremlægger og diskuterer forfatteren resultaterne af et forskningsprojekt, der under delprojektoverskriften ”Faglige diskussioner i computerkonferencer” er en del af forskningsprojektet ”eLearning i universitetsdidaktisk perspektiv - en undersøgelse af udvalgte teknologiers læringspotentiale”, som gennemføres af forskere på Danmarks Pædagogiske Universitet (DPU. I projektet ”Faglige diskussioner i computerkonferencer” undersøges det empirisk, hvordan masterstuderende anvender forhandling i computerkonferencer i forbindelse med deres individuelle læreprocesser. Fremstillingen i denne artikel relaterer sig således til temaet om erfaringer med feedback i netbaserede undervisningsforløb – her først og fremmest de studerendes gensidige feedback. I artiklen fremstiller forfatteren baggrunden for undersøgelsen, undersøgelsesfeltet, projektets teoretiske forankring og forskningsspørgsmål samt en operationalisering af forhandlingsbegrebet. Derefter fremlægges og diskuteres projektets resultater. -
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...
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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>
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Thermocron iButton and iBBat temperature dataloggers emit ultrasound.
Willis, Craig K R; Jameson, Joel W; Faure, Paul A; Boyles, Justin G; Brack, Virgil; Cervone, Tom H
2009-10-01
Thermocron iButton dataloggers are widely used to measure thermal microclimates experienced by wild animals. The iBBat is a smaller version of the datalogger, also commercially available, that is used to measure animal skin or core body temperatures when attached externally or surgically implanted. Field observations of bats roosting under a bridge suggested that bats avoided locations with iButtons. A heterodyne bat detector revealed that the dataloggers emitted ultrasound which was detectable from a distance of up to 30 cm. We therefore recorded and quantified the acoustic properties [carrier frequency (Hz) and root mean square sound pressure level (dB SPL)] of iButton and iBBat dataloggers. All units emitted a 32.9 kHz pure tone that was readily picked up with a time expansion bat detector at a distance of 1 cm, and most were detected at a distance of 15 cm. The maximum amplitude of iButton dataloggers was 46.5 dB SPL at 1.0 cm-a level within the range of auditory sensitivity for most small mammals. Wrapping iButtons in plastic insulation severely attenuated the amplitude of ultrasound. Although there was a statistically significant reduction in rates of warming and cooling with insulation, this effect was small and we suggest that insulation may be a viable solution to eliminate unwanted ultrasonic noise in instances when small delays in thermal response dynamics are not a concern. We recommend behavioural studies to assess if the electronic signals emitted by iButtons are disturbing to small mammals.
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Beredskapskunnskap – navet i Lærerutdanning i Kunst og håndverk
Directory of Open Access Journals (Sweden)
Marte Sørebø Gulliksen
2014-12-01
Full Text Available Concurrent (samtidig lærerutdanning møter en dobbelt utfordring: studentene skal lære et (eller flere fag, samtidig som de skal lære å lære bort fag. I Kunst og håndverk knyttes denne debatten ofte opp mot hvordan en i korte utdanningsenheter kan få nok faglig dybde i de ulike delene av faget. I artikkelens første del presenteres tidligere forskning på Kunst og håndverk. Et fagdidaktisk kunnskapsperspektiv trekkes opp i tilknytning til begrepet pedagogisk innholdskunnskap forstått som en re-forhandling av fagkunnskap og pedagogisk kunnskap (Shulman. Ut fra dette problematiseres fagets kvalifiseringsbredde og indre spenninger som en del av lærerutdanningens premiss. Det argumenteres for at en reduksjon av kvalifiseringsbredden ikke vil føre til redusert kompleksitet i undervisningen i faget. Artikkelens andre del foreslår begrepet beredskapskunnskap som en måte å håndtere fagets kompleksitet på: som et mulig grunnprinsipp for inn- og utvalg av arbeidsformer og innhold i utdanning i Kunst og håndverk. Diskusjonen knyttes til spørsmålet om hvordan lærerutdanning i Kunst og håndverk best kan ruste studenter til et langt liv som lærerprofesjonsutøvere i en fremtid vi ennå ikke vet hvordan ser ut. Artikkelen foreslår at beredskapskunnskap kan forstås som et nav i et prinsipp om livslang læring for lærere i Kunst og håndverk, og således som et overordnet mål for lærerutdanning generelt.
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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...
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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....
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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...
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Directory of Open Access Journals (Sweden)
Robert Sayre
2007-05-01
Full Text Available Trata-se de uma interpretação do romance <i>Gatsby, o Magníficoi>, de F. Scott Fitsgerald, a partir de aspectos essenciais da sociedade moderna, como a reificação e o consumo ostentatório, aspectos esses intimamente ligados e relativamente distintos. Trata-se, portanto de destacar algumas modalidades importantes de conhecimento <i>sui generisi> desses fenômenos, exclusivamente, pelos meios literários. Palavras-chave: Alienação. Classe ociosa. Reificação. Literatura. This paper is a sociological interpretation of the novel <I>The Great GatsbyI>, by F. Scott Fitsgerald, from the point of view of some of the essential aspects of modern society such as the reification and the boasting consume which are at the same time closely linked and relatively distinct. In fact, it tries to point out some important modalities of human knowledge through a literary form, the novel. Keywords: Alienation. Idle class. Literature. Reification.
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Business and research of the i phone and the i pad
Energy Technology Data Exchange (ETDEWEB)
NONE
2005-02-15
This is manual for the i phone and i pad, which instructs how to use them. It consists of eight parts and goes as follows; mail, contact like synchronizing of contact, know-how on application of contact and application technique for group business, schedule management about Google calendar, sharing calendar with others, Organizer, and application of Daum public calendar, utilizing document such as document tool, memo and writing on computer, news like watching news with a i phone and i pad, Google news and RSS, data management with safari, Dropbox, Good Reader and i Books, basic functions, map and weather and using iphone in foreign country.
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Business and research of the i phone and the i pad
International Nuclear Information System (INIS)
2005-02-01
This is manual for the i phone and i pad, which instructs how to use them. It consists of eight parts and goes as follows; mail, contact like synchronizing of contact, know-how on application of contact and application technique for group business, schedule management about Google calendar, sharing calendar with others, Organizer, and application of Daum public calendar, utilizing document such as document tool, memo and writing on computer, news like watching news with a i phone and i pad, Google news and RSS, data management with safari, Dropbox, Good Reader and i Books, basic functions, map and weather and using iphone in foreign country.
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Promjene na oralnoj sluznici i koži u trudnoći
Bukvić, Lana
2015-01-01
Trudnoća počinje usađivanjem oplođene jajne stanice u endometrij maternice. Traje deset lunarnih mjeseci ili 280 dana. Tijekom trudnoće nastaju brojne promjene u organizmu, kako fiziološke tako i patološke. Glavni razlog nastanka promjena je fluktuacija gestacijskih hormona. U usnoj šupljini je tijekom trudnoće čest nalaz gingivitisa s prevalencijom od 35 do 100%. Uz generaliziranu upalu, moguća je pojava i intenzivnije lokalne upale sluznice u obliku epulisa gravidaruma. Važno je zaustaviti ...
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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....
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Kan udvikling af strategier ændre undervisningskulturen i begynderundervisningen i matematik?
DEFF Research Database (Denmark)
Ejersbo, Lisser Rye
2014-01-01
Således indleder Dagmar Neuman en artikel, skrevet i et Nordisk matematikdidaktisk tidsskrift Nomad i 2013. På baggrund af observationer og interview med skoleelever beskriver hun, hvordan lærere kan komme matematikproblemer til livs gennem et paradigmeskifte i begynderundervisningen....
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Active learning electromagnetism with iPhone, iPod Touch and iPad: a new teaching resource
Arribas Garde, Enrique; Nájera López, Alberto; Beléndez Vázquez, Augusto; Francés Monllor, Jorge; Mora Ley, César Eduardo; Franco, María Teresa; Moratalla Mondéjar, Gonzalo
2012-01-01
The proliferation of so-called smart phones (among which we include the iPhone) and tablets (iPad and iPod Touch) is leading to these devices to be focused by teachers and researchers. Their power of calculation and their graphic capabilities make them to be extremely attractive for trying to increase the process of teaching and learning in a highly significant manner. Smart phones and other similar devices provide new ways of learning that we believe it is necessary to explore. They are also...
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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...
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International Nuclear Information System (INIS)
Frechou, C.
2000-01-01
129 I, is a natural long-lived isotope, with a half-life of 15,7 million years, also artificially produced in nuclear power plant. It is then released in the liquid and gaseous effluents of the nuclear fuel reprocessing plants. 129 I is integrated in all biological compartments at different activity levels, depending on their distance from the emission source and their ability to metabolize iodine. Performances of the different 129 I and 129 I/ 127 I measurement techniques available: Radiochemical Neutron Activation Analysis, Accelerator Mass Spectrometry, direct γ-X spectrometry and liquid scintillation were evaluated. Associated radiochemical preparation steps of the two first techniques were optimized and adapted to the characteristics of the major environmental matrices. In a first step, the radiochemical protocols were developed and validated. In a second step, intercomparison exercises have been lead on various environmental samples presenting different 129 I activity levels. They showed the good agreement between the results given by the three techniques on different environmental matrices with activities between 0,2 and 200 Bq.kg -1 dry weight. As a conclusion, a methodology for the measurement of 129 I and 129 I/ 127 I ratio in environmental samples is proposed. It includes a decisional diagram taking into account the characteristics of the matrices, the detection limits and the answer delay. A study on the losses of 129 I during the calcination of an algae was lead by direct γ-X spectrometry and application studies were made to measure 129 I levels in different biological compartments issued from various locations: 129 I activity interspecific variation in different species of seaweeds from the French channel coast under the relative influence of La Hague, 129 I levels in bovine thyroids from the Cotentin area and 129 I in vegetal samples collected around the nuclear reprocessing plant of Marcoule. (author)
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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...
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Directory of Open Access Journals (Sweden)
M. Conchillo
2007-03-01
Full Text Available El factor de crecimiento semejante a la insulina tipo I (IGF-I es una hormona polipeptídica segregada en múltiples tejidos por efecto de la hormona de crecimiento (GH. Es responsable de parte de las acciones de la GH y además tiene efecto hipoglucemiante y anabolizante. El 90% del IGF-I circulante es de origen hepático y ejerce efectos autocrinos, paracrinos y endocrinos, estos últimos en múltiples tejidos. En la cirrosis hepática se produce una disminución progresiva de la producción hepática de IGF-I que llega a ser indetectable en la enfermedad avanzada. Algunas de las complicaciones de la cirrosis, fundamentalmente nutricionales y metabólicas (resistencia a insulina, desnutrición, osteopenia, hipogonadismo, alteraciones intestinales podrían estar, al menos en parte, relacionadas con esta carencia de IGF-I dado que algunas acciones de IGF-I representan la imagen inversa de las complicaciones de la cirrosis. A pesar de ello, nunca se había propuesto tratamiento sustitutivo con IGF-I en la cirrosis. En una serie de estudios experimentales realizados en ratas cirróticas se demostró que el tratamiento con dosis bajas de IGF-I recombinante produce dos tipos de efectos en la cirrosis experimental: a mejoría del hígado, dado que mejora la función hepatocelular, la hipertensión portal y la fibrosis hepática; y b mejoría de las alteraciones extrahepáticas de la cirrosis dado que mejora la eficiencia del alimento ingerido, la masa muscular, la masa ósea, la función y estructura gonadales y la función y estructura intestinales con normalización de la malabsorción de azúcares y aminoácidos y la mejoría de la función intestinal de barrera manifestada por disminución de la endotoxemia y la translocación bacteriana. Posteriormente el primer ensayo clínico piloto, aleatorizado, doble ciego y controlado con placebo llevado a cabo en un número reducido de pacientes cirróticos demostró aumento de la albúmina sérica y
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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....
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Conversion of SMART I. Zur Konvertierung von SMART I
Energy Technology Data Exchange (ETDEWEB)
Argyris, J H; Szimmat, J; William, K J [Stuttgart Univ. (TH) (Germany, F.R.). Inst. fuer Statik und Dynamik der Luft- und Raumfahrtkonstruktionen
1977-01-01
The report describes the conversion of the programming system SMART I on CDC, UNIVAC and IBM computers under the BMFT grant RK 21 I/SBB 31. There were four tasks for the development of a machine-independent SMART-version: a) Updating of the CDC source library (ca. 180.000 Fortran statements); b) Conversion into double precision; c) Primary installation on UNIVAC; d) Primary installation on IBM. The conversion of the SMART I program was carried out in cooperation with the consulting firms RIB, Stuttgart, and IKOSS Stuttgart, under the leadership of ISD.
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Durzyńska, Julia; Philippou, Anastassios; Brisson, Becky K.; Nguyen-McCarty, Michelle
2013-01-01
IGF-I is a key regulator of muscle development and growth. The pre-pro-peptide produced by the Igf1gene undergoes several posttranslational processing steps to result in a secreted mature protein, which is thought to be the obligate ligand for the IGF-I receptor (IGF-IR). The goals of this study were to determine what forms of IGF-I exist in skeletal muscle, and whether the mature IGF-I protein was the only form able to activate the IGF-IR. We measured the proportion of IGF-I species in murine skeletal muscle and found that the predominant forms were nonglycosylated pro-IGF-I and glycosylated pro-IGF-I, which retained the C-terminal E peptide extension, instead of mature IGF-I. These forms were validated using samples subjected to viral expression of IGF-I combined with furin and glycosidase digestion. To determine whether the larger molecular weight IGF-I forms were also ligands for the IGF-IR, we generated each specific form through transient transfection of 3T3 cells and used the enriched media to perform kinase receptor activation assays. Compared with mature IGF-I, nonglycosylated pro-IGF-I had similar ability to activate the IGF-IR, whereas glycosylation of pro-IGF-I significantly reduced receptor activation. Thus, it is important to understand not only the quantity, but also the proportion of IGF-I forms produced, to evaluate the true biological activity of this growth factor. PMID:23407451
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Energy Technology Data Exchange (ETDEWEB)
Angeletos, T.; Londos, C. A., E-mail: hlontos@phys.uoa.gr [University of Athens, Solid State Physics Section, Panepistimiopolis Zografos, Athens 157 84 (Greece); Chroneos, A., E-mail: alexander.chroneos@imperial.ac.uk [Faculty of Engineering, Environment and Computing, Coventry University, Priory Street, Coventry CV1 5FB (United Kingdom); Department of Materials, Imperial College London, London SW7 2AZ (United Kingdom)
2016-03-28
Carbon-oxygen-self-interstitial complexes were investigated in silicon by means of Fourier transform infrared spectroscopy. Upon irradiation, the C{sub i}O{sub i} defect (C{sub 3}) forms which for high doses attract self-interstitials (Si{sub I}s) leading to the formation of the C{sub i}O{sub i}(Si{sub I}) defect (C{sub 4}) with two well-known related bands at 939.6 and 1024 cm{sup −1}. The bands are detectable in the spectra both in room temperature (RT) and liquid helium (LH) temperature. Upon annealing at 150 °C, these bands were transformed to three bands at 725, 952, and 973 cm{sup −1}, detectable only at LH temperatures. Upon annealing at 220 °C, these bands were transformed to three bands at 951, 969.5, and 977 cm{sup −1}, detectable both at RT and LH temperatures. Annealing at 280 °C resulted in the transformation of these bands to two new bands at 973 and 1024 cm{sup −1}. The latter bands disappear from the spectra upon annealing at 315 °C without the emergence of other bands in the spectra. Considering reaction kinetics and defect metastability, we developed a model to describe the experimental results. Annealing at 150 °C triggers the capturing of Si{sub I}s by the C{sub 4} defect leading to the formation of the C{sub i}O{sub i}(Si{sub I}){sub 2} complex. The latter structure appears to be bistable: measuring at LH, the defect is in configuration C{sub i}O{sub i}(Si{sub I}){sub 2} giving rise to the bands at 725, 952, and 973 cm{sup −1}, whereas on measurements at RT, the defect converts to another configuration C{sub i}O{sub i}(Si{sub I}){sub 2}{sup *} without detectable bands in the spectra. Possible structures of the two C{sub i}O{sub i}(Si{sub I}){sub 2} configurations are considered and discussed. Upon annealing at 220 °C, additional Si{sub I}s are captured by the C{sub i}O{sub i}(Si{sub I}){sub 2} defect leading to the formation of the C{sub i}O{sub i}(Si{sub I}){sub 3} complex, which in turn on annealing at
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Li I AND K I SCATTER IN COOL PLEIADES DWARFS
International Nuclear Information System (INIS)
King, Jeremy R.; Schuler, Simon C.; Hobbs, L. M.; Pinsonneault, Marc H.
2010-01-01
We utilize high-resolution (R ∼ 60,000), high signal-to-noise ratio (∼100) spectroscopy of 17 cool Pleiades dwarfs to examine the confounding star-to-star scatter in the λ6707 Li I line strengths in this young cluster. Our Pleiades, selected for their small projected rotational velocity and modest chromospheric emission, evince substantial scatter in the line strengths of λ6707 Li I feature that is absent in the λ7699 K I resonance line. The Li I scatter is not correlated with that in the high-excitation λ7774 O I feature, and the magnitude of the former is greater than the latter despite the larger temperature sensitivity of the O I feature. These results suggest that systematic errors in line strength measurements due to blending, color (or color-based T eff ) errors, or line formation effects related to an overlying chromosphere are not the principal source of Li I scatter in our stars. There do exist analytic spot models that can produce, via line formation effects, the observed Li scatter without introducing scatter in the K I line strengths or the color-magnitude diagram. However, these models predict factor of ≥3 differences in abundances derived from the subordinate λ6104 and resonance λ6707 Li I features; we find no difference in the abundances determined from these two features. These analytic spot models also predict CN line strengths significantly larger than we observe in our spectra. The simplest explanation of the Li, K, CN, and photometric data is that there must be a real abundance component to the Pleiades Li dispersion. We suggest that this real abundance component is the manifestation of relic differences in erstwhile pre-main-sequence Li burning caused by effects of surface activity on stellar structure. We discuss observational predictions of these effects, which may be related to other anomalous stellar phenomena.
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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....
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Den lille krig i fredstid. Danske militære erfaringer i Musa Qala i 2006
DEFF Research Database (Denmark)
Brink Rasmussen, Jakob
2018-01-01
Den 1. Lette Opklaringseskadron fra Bornholms forsvar af distriktscentret Musa Qala i den afghanske Helmand-provins i eftersommeren 2006 gennemanalyseres. Det sker med det formål at klarlægge, hvordan man udnyttede læring under forberedelsen til indsættelsen samt under og efter denne. Herunder da...
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Kritika važećeg i prijedlog novog pravnog uređenja vađenja i uklanjanja podrtina i potonulih stvari
Directory of Open Access Journals (Sweden)
Vesna Skorupan Wolff
2012-12-01
Full Text Available Glavni cilj rada je ponuditi i prikazati rješenja koja predstavljaju prijedlog novog pravnog uređenja instituta vađenja i uklanjanja podrtina i potonulih stvari. Prije toga, autorice u radu prikazuju genezu pravnih izvora u povijesti i ranijem domaćem zakonodavstvu te proučavaju sva relevantna pitanja i sve odredbe važećeg Pomorskog zakonika (u nastavku PZ o ovoj materiji. Analizira se značenje i uporaba pojedinih izraza, sistematizacija zakonske građe unutar strukture zakona te sadržaj i domašaj pojedinih odredbi. Autorice preispituju razinu usklađenosti pozitivnog PZ-a sa suvremenom međunarodnom regulativom u ovom području. Ukazuje se na važne probleme koji mogu nastati zbog manjkavosti odredbi pozitivnog PZ-a i nepostojanja sustavne regulacije svih relevantnih pitanja koja se mogu pojaviti u praksi. U okviru istraživanja provodi se i poredbena analiza ovih instituta u drugim nacionalnim zakonodavstvima. Predložena zakonska rješenja odlikuju se cjelovitošću i sustavnošću u normiranju svih relevantnih pitanja. Uređivanje ovih instituta specijalnim odredbama pružit će viši stupanj pravne sigurnosti te viši stupanj sigurnosti plovidbe Jadranom, zaštite okoliša, njegovih prirodnih bogatstava i drugih povezanih interesa.
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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...
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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...
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Mashhadiabbas, Fatemeh; Neamatzadeh, Hossein; Nasiri, Rezvan; Foroughi, Elnaz; Farahnak, Soudabeh; Piroozmand, Parisa; Mazaheri, Mahta; Zare-Shehneh, Masoud
2018-01-01
There has been increasing interest in the study of the association between Vitamin D receptor (VDR) gene polymorphisms and risk of chronic periodontitis. However, the results remain inconclusive. To better understand the roles of VDR polymorphisms (BsmI, TaqI, FokI, and ApaI) in chronic periodontitis susceptibility, we conducted this systematic review and meta-analysis. The PubMed, Google Scholar, and Web of Science database were systemically searched to determine all the eligible studies about VDR polymorphisms and risk of chronic periodontitis up to April 2017. Odds ratio (OR) and 95% confidence interval (CI) were used to evaluate the associations between VDR polymorphisms and chronic periodontitis risk. All the statistical analyses were performed by Comprehensive Meta-Analysis. All P values were two-tailed with a significant level at 0.05. Finally, a total of 38 case-control studies in 19 publications were identified which met our inclusion criteria. There are ten studies with 866 chronic periodontitis cases and 786 controls for BsmI, 16 studies with 1570 chronic periodontitis cases and 1676 controls for TaqI, five studies with 374 chronic periodontitis cases and 382 controls for FokI, and seven studies with 632 chronic periodontitis cases and 604 controls for ApaI. Overall, no significant association was observed between VDR gene BsmI, TaqI, FokI, and ApaI polymorphisms and risk of chronic periodontitis in any genetic model. Subgroup analysis stratified by ethnicity suggested a significant association between BsmI polymorphism and chronic periodontitis risk in the Caucasian subgroup under allele model (A vs. G: OR = 1.747, 95% CI = 1.099-2.778, P = 0.018). Further, no significant associations were observed when stratified by Hardy-Weinberg equilibrium status for BsmI, TaqI, and ApaI. Our results suggest that BsmI, TaqI, FokI, and ApaI polymorphisms in the VDR gene might not be associated with risk of chronic periodontitis in overall population.
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129I/127I ratios in surface waters of the English Lake District
International Nuclear Information System (INIS)
Atarashi-Andoh, M.; Schnabel, C.; Cook, G.; MacKenzie, A.B.; Dougans, A.; Ellam, R.M.; Freeman, S.; Maden, C.; Olive, V.; Synal, H.-A.; Xu, S.
2007-01-01
Accelerator Mass Spectrometry (AMS) was used to measure 129 I/ 127 I ratios in surface sea, lake, and river water samples collected in 2004 and 2005 from the English Lake District and from SW Scotland, areas which are in relatively close proximity to the Sellafield nuclear fuel reprocessing plant in NW England. The 129 I/ 127 I ratios in surface water collected from the shore of the Irish Sea were in the range 2.8 x 10 -6 to 8.2 x 10 -6 . These ratios are one order of magnitude higher than that of seawater collected from the Irish Sea in 1992, correlating with the increase in 129 I content of the Sellafield liquid effluent discharge over the last decade. The 129 I/ 127 I ratios in lakes in the Lake District were in the range 0.7 x 10 -6 to 6.4 x 10 -6 and decreased exponentially as a function of distance from Sellafield. Consideration of the relative variation of stable I concentrations and 129 I/ 127 I ratios suggests that Sellafield gaseous discharges may be the dominant source of 129 I to the lakes
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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. ...
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Directory of Open Access Journals (Sweden)
Halil IŞILAK
2015-07-01
Full Text Available In the Shia, social position of Shiite cleric on behalf of Imam, advanced gradually and it expanded to cover the majority of his authority. But this did not have systematic until the middle 19th cntury. In the 19th, instution of marja - i taqlid was systematized and it was thought that Shia cleric , except precidency , should assume the duties and responsibilities under the authority of the Imam and all members of the Shia should be follower of one of the cleric. Up to 1970’s marja - i taqlids thought about political theory and devoloped a theory on the ideal form of go vernmend fort he Shiite despite of Imam’s occultation. But in the period of Imam’s occultation they did not suggest that Imam’s presidential authorty should be assumed by Shia cleric. In the 1970’s Khomeini suggested that Shiite cleric should assume the Im am’s presidential authorty. In the 1979 Iranian Islamic Revolution took place and Kkomeini has implemented the theory of vilayet - i faqih . From this date, institution of marja - ı taqlid and concept of vilayet - i faqih began to pose contradictions. Majority of the marja - ı taqlids opposed the theory of theory of vilayet - i faqih and they stated that there is no such presidential authorty on behalf of the Imam. Muhsin al - Hakim, was the most important religious authority in Nacaf in this period, opposed to the vie ws of Khomeini. However, after the 1979 revolution the most important and powerful opposition to vilayet - i faqih shown by Abul Qasim Khui in Iraq.
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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...
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International Nuclear Information System (INIS)
Johnston, T.Y.
1985-01-01
On most computer systems the most common cause of performance degradation is I/O contention. This paper will examine some efforts that can be taken in a VM environment to reduce I/O or its effect at both the global and local levels
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La revista <i>Arxiu d´Etnografia de Catalunyai>: una retrospectiva
Directory of Open Access Journals (Sweden)
Bodoque Puerta, Yolanda
2002-06-01
Full Text Available <i>Arxiu d'Etnografia de Catalunyai> is one of the most influential journals in Spain in the field of social and cultural anthropology. Published by researchers working in the Division of Social Anthropology of the University Rovira i Virgili of Tarragona, <i>AEC> boasts a repertoire of monographic issues, fieldwork guides and bibliographies which are mandatory reading for both the national scientific community and foreign scholars interested in Spain.
El <i>Arxiu d'Etnografia de Catalunyai> es una de las revistas especializadas en antropología socio-cultural más influyentes en España desde su nacimiento. Publicada por los investigadores del Área de Antropología social de la Universidad Rovira i Virgili (Tarragona, sus dossiers monográficos, las guías de trabajo y las recopilaciones bibliográficas que ha publicado son de obligada referencia para la comunidad científica nacional y los investigadores foráneos interesados en España. -
Overvågning af aviær influenza i vilde fugle i Danmark 2016
DEFF Research Database (Denmark)
Hjulsager, Charlotte Kristiane; Krog, Jesper Schak; Madsen, Jesper J.
H5 eller H7 subtype, påvist i en stormmåge. De øvrige 138 prøver var negative for AI virus. HPAI H5N8 blev også påvist i en hobby besætning i november 2016. Fund af HPAI H5N8 i vilde fugle fortsatte i 2017. Molekylærfylogenetisk analyse af HA genet i HPAI H5N8 virus fundet i Danmark i 2016 viste...
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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...
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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...
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Overvågning af aviær influenza i vilde fugle i Danmark 2014
DEFF Research Database (Denmark)
Hjulsager, Charlotte Kristiane; Krog, Jesper Schak; Madsen, Jesper J.
Overvågningen af aviær influenza (AI) virus i vilde fugle i Danmark i 2014 blev udført i samarbejde mellem Veterinærinstituttet, Danmarks Tekniske Universitet (DTU-VET), Fødevarestyrelsen (FVST) og Statens Naturhistoriske Museum, Københavns Universitet (SNM) i henhold til ” Projektplan vedr....... overvågningen af aviær influenza i vilde fugle i Danmark i 2014” (bilag 4). Overvågning af AI på EU niveau går tilbage til 2002, og Danmark er underlagt EU kommissionens bestemmelser for udformning af overvågningen, der dog har skiftet gennem årene i takt med indhøstede erfaringer. I 2014 blev der udført passiv...
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Revisión taxonómica de <i>Alytes grandisi> Brunner (Amphibia, Anura
Directory of Open Access Journals (Sweden)
Sanchiz, B.
1986-12-01
Full Text Available The holotype and only existing remain referred to <i>Alytes grandisi> Brunner, 1957, presumably a discoglossid frog from the German Pleistocene, is examined and compared with other living and fossil Palaearctic anurans. The analysis rejects its validity as an independent extinct species, as The material is clearly within the known variability of living <i>Rana temporaria Linnaeusi>, 1758, of which it should be considered merely a sinonym.
Se examina el holotipo y único resto atribuido de <i>Alytes grandisi> Brunner, 1957, un supuesto discoglósido del Pleistoceno alemán, comparándose con otros anuros actuales y fósiles del Paleártico. El análisis permite desechar su pertenencia a una especie extinta independiente, siendo en cambio atribuible con toda claridad a la viviente <i>Rana temporaria Linnaeusi>, 1758, de la que debe considerarse sinónimo.
Zum systematischen Status von Alytes grandis Brunner, 1957 (Amphibia, Anura. Der bislang einzig bekannte und von Brunner (1957 beschriebene Rest zu <i>Alytes grandisi> aus pleistozänen Ablagerungen der Breitenberghöhle bei Gössweinstein (Fränk, Alb wird beschrieben und aufgrund ausführlicher Vergleichsuntersuchungen der rezenten Art <i>Rana temporaria Linnaeusi>, 1758, zugeordnet, <i>Alytes grandisi> Brunner, 1957, ist somit als synonyrn zu <i>Rana temporariai> zu betrachten. -
On Fuzzy β-I-open sets and Fuzzy β-I-continuous functions
International Nuclear Information System (INIS)
Keskin, Aynur
2009-01-01
In this paper, first of all we obtain some properties and characterizations of fuzzy β-I-open sets. After that, we also define the notion of β-I-closed sets and obtain some properties. Lastly, we introduce the notions of fuzzy β-I-continuity with the help of fuzzy β-I-open sets to obtain decomposition of fuzzy continuity.
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On Fuzzy {beta}-I-open sets and Fuzzy {beta}-I-continuous functions
Energy Technology Data Exchange (ETDEWEB)
Keskin, Aynur [Department of Mathematics, Faculty of Science and Arts, Selcuk University, Campus, 42075 Konya (Turkey)], E-mail: akeskin@selcuk.edu.tr
2009-11-15
In this paper, first of all we obtain some properties and characterizations of fuzzy {beta}-I-open sets. After that, we also define the notion of {beta}-I-closed sets and obtain some properties. Lastly, we introduce the notions of fuzzy {beta}-I-continuity with the help of fuzzy {beta}-I-open sets to obtain decomposition of fuzzy continuity.
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Skolestøtte til børn i familiepleje – delrapport I
DEFF Research Database (Denmark)
Eiberg, Misja; Andersen, Luna Kragh; Scavenius Sonne-Schmidt, Christoffer
I denne rapport beskrives resultaterne af et randomiseret kontrolleret forsøg med to skolestøttende indsatser til familieplejeanbragte børn i den danske folkeskole i alderen 6-14 år. Gennem afprøvningen af de to indsatser har forskningsprojektet ”Skolestøtte til børn i familiepleje” undersøgt, hv...
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25 CFR 26.33 - How do I show I need job training?
2010-04-01
... 25 Indians 1 2010-04-01 2010-04-01 false How do I show I need job training? 26.33 Section 26.33 Indians BUREAU OF INDIAN AFFAIRS, DEPARTMENT OF THE INTERIOR HUMAN SERVICES JOB PLACEMENT AND TRAINING PROGRAM Training Services § 26.33 How do I show I need job training? The need for Job Placement and...
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DEFF Research Database (Denmark)
Obara, Isaiah; Nielsen, Morten; Jeschek, Marie
2016-01-01
There is strong evidence that the immunity induced by live vaccination for control of the protozoan parasite Theileria parva is mediated by class I MHC-restricted CD8+ T cells directed against the schizont stage of the parasite that infects bovine lymphocytes. The functional competency of class I...... with peptides. In silico functional analysis resulted in peptide binding specificities that were largely distinct between the two breeds. We also demonstrate that CD8+ T cells derived from Ankole cattle that are seropositive for T. parva do not recognize vaccine candidate antigens originally identified...
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Metformina – mechanizmy działania i zastosowanie w terapii cukrzycy typu 2[i][/i
Directory of Open Access Journals (Sweden)
Marzena Grzybowska
2011-01-01
Full Text Available Metformina jest obecnie najczęściej zalecanym lekiem w terapii cukrzycy typu 2. Mimo iż ta pochodna biguanidu jest stosowana od ponad 50 lat, mechanizm jej działania nie został dokładnie poznany. W pracy przedstawiono najnowsze doniesienia o mechanizmach antyhiperglikemicznego działania metforminy. Obejmują one: zmniejszenie wchłaniania glukozy w jelicie cienkim, zwiększony transport glukozy do komórek, obniżenie osoczowego stężenia wolnych kwasów tłuszczowych oraz hamowanie glukoneogenezy. Szczególną rolę w tych procesach odgrywa aktywacja kinazy białkowej aktywowanej przez AMP. Najnowsze odkrycia umożliwiły poznanie mechanizmów działania przeciwmiażdżycowego, hipotensyjnego i przeciwnowotworowego metforminy oraz jej wpływu na czynność śródbłonka naczyń. Plejotropowe działanie metforminy obejmuje wpływ na profil lipidowy osocza, zmniejszenie stresu oksydacyjnego, a także zwiększenie aktywności fibrynolitycznej osocza. Mimo że metformina nie jest metabolizowana, najnowsze badania wykazały, że jest aktywnie transportowana do hepatocytów, a także do komórek nabłonka kanalików nerkowych, odpowiednio przez OCT1 (organic cation transporter 1, kodowany przez gen SLC22A1 oraz OCT2 (kodowany przez [i]SLC22A2[/i]. Z kolei transporter MATE1 (multidrug and toxin extrusion 1 protein, kodowany przez gen [i]SLC47A1[/i] umożliwia wydzielanie metforminy z tych komórek do żółci lub moczu. Polimorfizm genów transporterów metforminy może się przyczynić do istotnych różnic w reakcji na lek.
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Clinical uses of I-123 produced by 127I(p, 5n)123Xe to 123I reaction in NIRS
International Nuclear Information System (INIS)
Saegusa, Kenji; Arimizu, Noboru; Uchiyama, Guio; Tateno, Yukio; Rikitake, Tomoyuki.
1978-01-01
123 I capsules produced by NIRS which are believed to be uncontaminated by radioactive impurities other than 125 I were compared with commercial 123 I capsules regarding gamma-ray spectra, thyroid phantoms and clinical scintigrams. Absorbed radiation doses of 123 I contaminated with nuclides other than 123 I to thyroid and whole body were also estimated. Regarding gamma-ray spectra, any nuclides other than 125 I(0.53%) did not contaminate in 123 I produced by NIRS, and it was superior to commercial capsules. Regarding phantoms and clinical scintigrams, background counts around the thyroid gland seemed to be slightly higher in commercial capsules than that produced by NIRS because of contamination with other nuclides. Exposed doses in thyroid and whole body were counted. Ratios in thyroid and whole body were increased by 30% and 9%, respectively in 123 I produced by NIRS because of contamination with 0.53% of 125 I in the event that the intake ratio of thyroid was determined as 25%. In commercial capsules the doses in thyroid and whole body were increased by 500% and 150%, respectively. Doses of commercial capsules and NIRS capsules were 7.87 rad and 1.72 rad, respectively per 100 μCi in thyroid. The ratio of NIRS capsules to commercial capsules in thyroid was 1/4.6, and that in the whole body was less than 1/2. (Ichikawa, K.)
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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
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Reproducibility of [123I]PE2I binding to dopamine transporters with SPECT
DEFF Research Database (Denmark)
Ziebell, Morten; Thomsen, Gerda; Knudsen, Gitte M
2007-01-01
The iodinated cocaine derivative [(123)I]PE2I is a new selective ligand for in vivo studies of the dopamine transporter (DAT) with SPECT. Recently, a bolus/infusion (B/I) protocol for [(123)I]PE2I measurements of DAT density was established [Pinborg LH et al. J Nucl Med 2005;46:1119-271]. The aims...
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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...
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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...
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Angiotensin-I converting enzyme gene and I/D polymorphism ...
Indian Academy of Sciences (India)
Angiotensin-I converting enzyme gene and I/D polymorphism distribution in the Greek population and a comparison with other European populations. Sekerli Eleni Katsanidis Dimitrios Papadopoulou Vaya Makedou Areti Vavatsi Norma Gatzola Magdalini. Research Note Volume 87 Issue 1 April 2008 pp 91-93 ...
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Properties of CsI and CsI-TMAE photocathodes
International Nuclear Information System (INIS)
Anderson, D.F.; Kwan, S.; Peskov, V.; Hoeneisen, B.
1992-06-01
The importance of heating the CsI or CsI-TMAE photocathodes during preparation, as well as the importance of the gas environment on the quantum efficiency is presented. The dependence of the aging characteristics of these photocathodes on the operating temperature, on the presence of gas, and on the charge amplification of the chamber is also discussed. For CsI photocathodes charges in excess of 2x10 14 e - /mm 2 can be collected with little degradation of performance. A timing resolution of 0.55 ns is also achieved for single photoelectrons suggesting a possible time-of-flight detector
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Pećnik, Ninoslava; Matić, Jelena; Tokić Milaković, Ana
2016-01-01
Europske obitelji proživljavaju promjene u pogledu odnosa moći između članova obitelji, uključujući demokratizaciju odnosa između roditelja i djece. Te je procese olakšala primjena Konvencije o pravima djeteta UN-a (1989.) koju je Hrvatska ratificirala 1991. godine. Ova studija analizira percipiranu realizaciju prava djeteta na skrb, zaštitu i participaciju unutar modernih hrvatskih obitelji. Osim toga, istražuje veze između realizacije participatornih prava i dječje percepcije demokratske kl...
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The preparation of 125I - gastrin I and 125 - minigastrin for medical diagnostics
International Nuclear Information System (INIS)
Byszewska-Szpocinska, E.; Markiewicz, A.
2002-01-01
Gastrin I G-17 and minigastrin G-13 were iodinated using direct methods with chloramin T and iodogen procedures and by indirect method with Bolton-Hunter reagent. 125 I - gastrin and 1 25 I - minigastrin were isolated from the iodination mixtures by gel filtration on Sephadex G-10 and Sephadex G-25 (PD-10 column) and by HPLC system (Lichrospher WP-300-RP-18 column). Radiochemical purity was shown by HPLC also. It was confirmed that iodogen procedure is the best for iodination of these peptides. 125 I - gastrin I obtained by this method with specific activity 80 μCi/nmol was homogeneous but iodination to higher specific activity (440μCi/nmol) caused appearance of two subfractions. 125 I - minigastrin with high and low specific activity were isolated by HPLC as the two forms (subfractions). It was shown that high performance liquid chromatography (HPLC) was the best method for isolation and purification of 125 I - gastrin I and 125 I - minigastrin. (author)
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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,...
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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...
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Overvågning af aviær influenza i vilde fugle i Danmark 2013
DEFF Research Database (Denmark)
Hjulsager, Charlotte Kristiane; Krog, Jesper Schak; Madsen, Jesper J.
Overvågningen af aviær influenza (AI) virus i vilde fugle i Danmark i 2012 er udført i samarbejde mellem Fødevarestyrelsen (FVST), Veterinærinstituttet, Danmarks Tekniske Universitet (DTU-VET) og Statens Naturhistoriske Museum, Københavns Universitet (SNM) i henhold til ”Aftale mellem Fødevaresty......Overvågningen af aviær influenza (AI) virus i vilde fugle i Danmark i 2012 er udført i samarbejde mellem Fødevarestyrelsen (FVST), Veterinærinstituttet, Danmarks Tekniske Universitet (DTU-VET) og Statens Naturhistoriske Museum, Københavns Universitet (SNM) i henhold til ”Aftale mellem...... Fødevarestyrelsen og Veterinærinstituttet om overvågning af aviær influenza i vilde fugle i Danmark i 2012” (bilag 4). Overvågning af AI på EU niveau går tilbage til 2002, og Danmark er underlagt EU kommissionens bestemmelser for udformning af overvågningen, der dog har skiftet gennem årene i takt med indhøstede...... erfaringer. I 2012 blev der udført passiv overvågning af døde vilde fugle, der blev fundet i naturen på lokaliteter, der fordelte sig over det meste af landet. Der blev testet 18 fugle, og ingen af disse var positive for HPAI. To prøver var positive for LPAI virus, men disse virus var ikke af influenza A H5...
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Overvågning af aviær influenza i vilde fugle 2012 i Danmark
DEFF Research Database (Denmark)
Hjulsager, Charlotte Kristiane; Trebbien, Ramona; Madsen, Jesper J.
Overvågningen af aviær influenza (AI) virus i vilde fugle i Danmark i 2012 er udført i samarbejde mellem Fødevarestyrelsen (FVST), Veterinærinstituttet, Danmarks Tekniske Universitet (DTU-VET) og Statens Naturhistoriske Museum, Københavns Universitet (SNM) i henhold til ”Aftale mellem Fødevaresty......Overvågningen af aviær influenza (AI) virus i vilde fugle i Danmark i 2012 er udført i samarbejde mellem Fødevarestyrelsen (FVST), Veterinærinstituttet, Danmarks Tekniske Universitet (DTU-VET) og Statens Naturhistoriske Museum, Københavns Universitet (SNM) i henhold til ”Aftale mellem...... Fødevarestyrelsen og Veterinærinstituttet om overvågning af aviær influenza i vilde fugle i Danmark i 2012” (bilag 4). Overvågning af AI på EU niveau går tilbage til 2002, og Danmark er underlagt EU kommissionens bestemmelser for udformning af overvågningen, der dog har skiftet gennem årene i takt med indhøstede...... erfaringer. I 2012 blev der udført passiv overvågning af døde vilde fugle, der blev fundet i naturen på lokaliteter, der fordelte sig over det meste af landet. Der blev testet 18 fugle, og ingen af disse var positive for HPAI. To prøver var positive for LPAI virus, men disse virus var ikke af influenza A H5...
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Treatment of lysosomal storage disease in MPS VII mice using a recombinant adeno-associated virus.
Watson, G L; Sayles, J N; Chen, C; Elliger, S S; Elliger, C A; Raju, N R; Kurtzman, G J; Podsakoff, G M
1998-12-01
Mucopolysaccharidosis type VII (MPS VII) is a lysosomal storage disease caused by a genetic deficiency of beta-glucuronidase (GUS). We used a recombinant adeno-associated virus vector (AAV-GUS) to deliver GUS cDNA to MPS VII mice. The route of vector administration had a dramatic effect on the extent and distribution of GUS activity. Intramuscular injection of AAV-GUS resulted in high, localized production of GUS, while intravenous administration produced low GUS activity in several tissues. This latter treatment of MPS VII mice reduced glycosaminoglycan levels in the liver to normal and reduced storage granules dramatically. We show that a single administration of AAV-GUS can provide sustained expression of GUS in a variety of cell types and is sufficient to reverse the disease phenotype at least in the liver.
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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...
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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....
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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...
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Binding of IGF I and IGF I-stimulated phosphorylation in canine renal basolateral membranes
International Nuclear Information System (INIS)
Hammerman, M.R.; Gavin, J.R. III.
1986-01-01
To characterize the interaction of the renal proximal tubular cell with insulin like growth factor I (IGF I), we measured binding of 125 I-IGF I to proximal tubular basolateral membranes from dog kidney and induced IGF I-stimulated phosphorylation of basolateral membranes. Specific binding of 125 I-IGF I to basolateral membranes was observed that was half-maximal at between 10(-9) and 10(-8) M IGF I. 125 I-IGF I was affinity cross-linked to a 135,000 Mr protein in basolateral membranes that was distinct from the alpha-subunit of the insulin receptor and from the IGF II receptor. IGF I-stimulated phosphorylation of a 92,000 Mr protein was effected in detergent-solubilized membranes incubated with 100 microM [gamma- 32 P]ATP. The 32 P-labeled protein was distinct from the beta-subunit of the insulin receptor, the 32 P phosphorylation of which was stimulated by insulin. We conclude that specific receptors for IGF I are present in the basolateral membrane of the renal proximal tubular cell. Physiological actions of IGF I at this nephron site may occur through the binding of this peptide circulating in plasma, to specific basolateral membrane receptors, followed by IGF I stimulated phosphorylation
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Arabic literary elements in the structure of the <i>Libro de buen amori>
Directory of Open Access Journals (Sweden)
Monroe, James T.
2011-06-01
Full Text Available This article examines several aspects of Arab-Islamic culture, about which Juan Ruiz, Archpriest of Hita, and putative author of the <i>Libro de buen amori>, betrays evidence of some knowledge. It goes on to suggest that, while the <i>LBA`s> literary <i>materials> are largely of Western origin, its <i>structure> is indebted to a unique combination of certain Oriental literary genres, thereby making it a culturally hybrid work. The article further suggests that the <i>LBA> may be read according to three levels of meaning, in accordance with the Averroistic approach to, and conception of, truth, of which the highest, or philosophical level, involves a veiled critique of the doctrine of ecclesiastical celibacy, adopted by the Western Church only a century before the Archpriest composed his work
En este artículo se examinan algunos aspectos de la cultura arabigoislámica, de los cuales Juan Ruiz, Arcipreste de Hita, presunto autor del <i>Libro del Buen Amori>, demuestra poseer cierto conocimiento. De resultas se sugiere que, mientras los <i>materiales> literarios constituyentes del <i>Libro> son de raigambre occidental, su <i>estructura> se debe a una combinación de ciertos géneros literarios orientales, de lo cual el <i>Libro> resultaría ser una obra culturalmente híbrida. Además, se sugiere que el libro debe interpretarse según tres niveles de significado, de acuerdo con la metodología desarrollada por Averroes para captar la verdad, y que, de esos tres niveles, el más elevado, a saber , el filosófico, nos conduce a una crítica velada de la doctrina del celibato eclesiástico, adoptada por la iglesia occidental solo un siglo antes de la época en que el Arcipreste escribiera su obra. -
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....
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Chemical speciation analysis and environmental behaviour of 127I and 129I
Energy Technology Data Exchange (ETDEWEB)
Hansen, Violeta
2011-10-15
Chemical speciation analysis of 129I and 127I as iodide, iodate and total inorganic iodine in seawater samples from the Baltic Proper, Skagerrak and Kattegat has been carried out. The important findings of this study are that the reduction of iodate and oxidation of iodide in Skagerrak and Kattegat may be a slow process while along the Baltic Sea surface water reduction of iodate is a relatively fast process. Although suboxic or anoxic condition are encountered in some of the Baltic Sea deep basins, the concentration of 129IO{sub 3}- increases with water depth indicating that the reduction of iodate in the oxygen deficient bottom water of the Baltic Sea is a slow process. Iodine chemical speciation analysis (as iodide, iodate and total iodine including inorganic and organic iodine species) in lake water samples collected from Denmark and southern Sweden has been carried out. Destruction of organic iodine was performed by alkaline oxidation using NaOH - NaClO at 100 deg. C and anion exchange chromatography was used for separation of iodide and iodate. Iodine-129 concentrations in the lakes ranged from 1.3 - 12.8 x109 at/L and show elevated concentrations in lakes located in southwest Jutland (Denmark), near the North Sea. Except the Skaersoe Lake, were the organic iodine - 127 accounts for 50% of the total iodine, iodide (both 129I and 127I) is the predominant species form in surface water of the studied lakes. An investigation was conducted in order to quantify the total aquatic iodine (129I and 127I as inorganic and organic iodine) from fresh water and seawater samples by adsorption onto activated charcoal and DEAE 32 cellulose followed by alkaline digestion or combustion. The results show that iodide from freshwater samples can easily be adsorbed onto activated charcoal. The sorption was not affected by the pH. The absorption capacity of iodate is low and reduces quickly when its concentration increases. Compared with activated charcoal, DEAE 32 cellulose showed
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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.
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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....
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Radiation damage in undoped CsI and CsI(Tl)
International Nuclear Information System (INIS)
Woody, C.L.; Kierstead, J.A.; Levy, P.W.; Stoll, S.
1992-01-01
Radiation damage has been studied in undoped CsI and CsI(TI) crystals using 60 Co gamma radiation for doses up to ∼ 4.2 x 10 6 . Samples from various manufacturers were measured ranging in size from 2.54 cm long cylinders to a 30 cm long block. Measurements were made on the change in optical transmission and scintillation light output as a function of dose. Although some samples showed a small change in transmission, a significant change in light output was observed for all samples. Recovery from damage was also studied as a function of time and exposure to UV light. A short lived phosphorescence was observed in undoped CsI, similar to the phosphorescence seen in CsI(TI)
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Energy Technology Data Exchange (ETDEWEB)
Osterc, Andrej [Department of Environmental Sciences, Jozef Stefan Institute, Jamova 39, 1000 Ljubljana (Slovenia); Stibilj, Vekoslava [Department of Environmental Sciences, Jozef Stefan Institute, Jamova 39, 1000 Ljubljana (Slovenia)], E-mail: vekoslava.stibilj@ijs.si
2008-04-15
The only stable iodine isotope is {sup 127}I and the natural {sup 129}I/{sup 127}I ratio in the biosphere has increased from 10{sup -15}-10{sup -14} to 10{sup -10}-10{sup -9}, mainly due to emissions from nuclear fuel reprocessing plants. In Europe they are located at La Hague (France) and Sellafield (England), where the ratio of {sup 129}I/{sup 127}I is up to 10{sup -4}. The marine environment, i.e. the oceans, is the major source of iodine with average concentrations of around 60 {mu}g L{sup -1} iodine in seawater. Brown algae accumulate iodine at high levels of up to 1.0% of dry weight, and therefore they are an ideal bioindicator for studying the levels of {sup 127}I and {sup 129}I in the marine environment. A radiochemical neutron activation analysis (RNAA) method, developed at our laboratory, was used for {sup 129}I determination in the brown alga Fucus virsoides (Donati) J. Agardh, and the same technique of RNAA was used for total {sup 127}I determination. The samples were collected along the coast of the Gulf of Trieste and the West coast of Istria in the North Adriatic Sea in the period from 2005 to 2006. Values of the {sup 129}I/{sup 127}I ratio up to 10{sup -9} were found, which is in agreement with the present average global distribution of {sup 129}I. The levels of stable iodine found were in the range from 235 to 506 {mu}g g{sup -1} and the levels of {sup 129}I from 1.7 to 7.3 x 10{sup -3} Bq kg{sup -1} (2.6-10.9 x 10{sup -7} {mu}g g{sup -1}), on a dry matter basis.
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Energy Technology Data Exchange (ETDEWEB)
NONE
1963-12-15
Cooperation between the Institutes in Vinca and Saclay has started by carrying out the task 'Technical realization of the VISA-2 project' which should enable: obtaining new experimental spaces in the RA reactor with high fast neutron flux for sample irradiation; obtaining experience in reactor continual operation for few months at nominal power of 6.5 MW and increase of RA reactor utilization; solving the problem of activated channels and samples transport, problems of working in hot cells; obtaining irradiated samples for examining radiation effects first in Saclay and later in Vinca. The project is divided in three phases. Phase one covers the reconstruction of the RA reactor fuel channels according to the VISA-2 project demands. The second phase includes the activities related to measuring devices for measuring the temperatures at the incoming and outgoing heavy water in 5 VISA-2 channels, as well as temperature of the samples (55 thermocouples) and testing the channels and capsules after their insertion in the reactor. The third phase includes activities are related to problems of transport of radioactive channels and VISA-2 capsules, problems of cutting as well as packing and transporting of irradiated samples from Vinca to Saclay. This volume includes all the relevant documents for completing the task including contracts, needed preliminary calculations as well as safety analysis. [Serbo-Croat] Saradnja izmedju Instituta u Vinci i Saclay-u zapoceta je realizacijom zadatka 'Tehnicka realizacije projekta VISA-2' koji treba da omoguci: dobijanje novih eksperimentalnih prostora u reaktoru RA sa visokom vrednoscu fluksa brzih neutrona za ozracivanje uzoraka; sticanje pogonskih iskustava u visemesecnom kontilualnom radu na nominalnoj snazi od 6,5 MW i povecanje stepena koriscenja reaktora RA; resenje pitanja transporta aktivnih kanala i uzoraka, problema rada u vrucim celijama; dobijanje ozracenih uzoraka za izucavanje, u prvo vreme u Saclay-u, a kasnije i u Vinci
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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...
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Directory of Open Access Journals (Sweden)
Iwona Mozer-Lisewska
2011-08-01
Full Text Available The aim of this study was to provide evidence for the expression of iNOS in the cells of inflammatory infiltrates around larvae in skeletal muscles of T. spiralis infected mice. The BALB/c mice (n=8 divided into subgroups, received either aminoguanidine (AMG - a specific iNOS inhibitor or albendazole (ALB - an antiparasitic drug of choice in trichinellosis treatment. Control animals (n=2 in each subgroup were either uninfected and treated or uninfected and untreated. Frozen sections of hind leg muscles from mice sacrificed at various time intervals after infection were cut and subjected to immunohistochemistry, using monoclonal anti-iNOS antibody. The ALB-treated mice revealed stronger iNOS staining in the infiltrating cells around larvae than the infected and untreated animals. On the contrary, in the AMG-treated animals, the infiltrating cells did not show any specific iNOS reaction. These data confirm the specificity of iNOS staining in the cellular infiltrates around <i>T. spiralisi> larvae and shed some light on the role of nitric oxide during ALB treatment in experimental trichinellosis.
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International Nuclear Information System (INIS)
Birchall, T.; Denes, G.; Faggiani, R.; Frampton, C.S.; Gillespie, R.J.; Kapoor, R.; Vekris, J.E.
1990-01-01
Iodine is oxidized by peroxodisulfuryl difluoride, S 2 O 6 F 2 , to give I(OSO 2 F) 2 I. The crystal structure of the orthorhombic type crystal is reported. The structure was solved by means of Patterson functions and refined by least squares to final agreement indices of R 1 = 0.0353 and R 2 = 0.0374 for 1,600 independent reflections. There are three primary bonds to the central iodine, I(1), (I(1)-OSO 2 F = 2.086 (7) and 2.258 (7) angstrom; I(1)-I(2) = 2.676 (1) angstrom), which create a distorted T=shaped AX 3 E 2 geometry. The second iodine, I(2), has a primary bond to I(1) and a strong intermolecular secondary I(2)-O bond of length 2.655 (8) angstrom to one of the fluorosulfate groups that is colinear with the primary bond, giving an AXYE 3 geometry about I(2). The Raman spectrum of the solid and the 127 I Moessbauer spectrum are in full agreement with the structure found. 30 refs., 3 figs., 4 tabs
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Tıpta işbirliği ve hukuksal sorunlar
Rosenau, Henning
2010-01-01
Tıpta işbirliği ve hukuksal sorunlar : tarih: 16-17 nisan 2010, Kurupelit Yerleskesi, Samsun = Delegation und Kooperation im Gesundheitswesen / ed.: Henning Rosenau ... - Samsun : Adalet, 2010. - XII, 331 S.
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Directory of Open Access Journals (Sweden)
Romero Sá, Magali
2010-06-01
Full Text Available This article presents the development of the journals <i>Revista Médica de Hamburgoi> and <i>Revista Médica Germano-Ibero-Americanai>, which were created to promote and disseminate the German science among the medical community in Latin America and Spain between the two World Wars. Shaken by the loss of Germany’s colonies in Africa, the difficulties faced due to post-war economy, and the restrictions imposed by the armistice, the Germans sought to restore their cultural and scientific prestige through such initiative.
El trabajo aborda la trayectoria de la <i>Revista Médica de Hamburgo (RMHi> y la <i>Revista Médica Germano-Ibero-Americana (RMGIAi>, publicaciones creadas para promover y difundir la ciencia alemana entre las comunidades médicas de América Latina y España en el contexto de entreguerras. Sacudidos por la pérdida de las colonias en África, por las dificultades enfrentadas por la economía y por las condiciones impuestas por el armisticio, los alemanes pretendían con esa iniciativa recuperar el prestigio cultural y científico. -
Cloisonné kidney in mouflon sheep (<i>Ovis orientalis musimoni>
Directory of Open Access Journals (Sweden)
Hervás, J.
2002-12-01
Full Text Available This study describes a case of cloisonné kidney associated with infection by <i>Babesia ovis i>in a wild mouflon (<i>Ovis orientalis musimoni>j found dead in year in eastern Sierra Morena (Andalusia, Spain. The major macroscopic lesions were a icteric coloration in ocular and oral mucosa, and b dark metal coloration on the kidneys' external surface. Intense dark coloration alternating with intense greyish strips was observed on renal cortex sections. Blood samples were collected for haematological and parasitological analyses, as well as samples from various organs for histopathological examination. Infection by <i>B. ovisi> was confirmed by using the Polymerase Chain Reaction test (PCR. Histopathological examination revealed tubulonephrosis associated with thickening of the basal membrane of the convoluted portions of the renal tubules (both proximal and distal, which took on an intense brownish pigmentation. These renal lesions have been described as "cloisonné" kidney. Cloisonné kidneys show uncommon coloring of the renal tubular system, associated with ferritin and hemosiderin deposits in these structures resulting from a chronic hemolitic process (hemoparasites, toxins, etc.. In our case, hemolitic phenomena are associated with infection by <i>B. ovisi>. The seroprevalence of this hemoparasite in wild mouflon populations in other regions of Spain (Catalonia is up to 15%.
[fr]
Nous étudions un cas de rein cloisonné associé à l'infection par <i>Babesia ovis i>sur un mouflon sauvage (<i>Ovis orientalis musimoni> qui a été trouvé la même année dans la partie orientale de la Sierra Morena (Andalousie, Espagne. Les principales lésions macroscopiques étaient: a coloration ictérique sur les muqueuses oculaire et orale, et b coloration métallique foncée sur la partie extérieure du rein. Cette coloration foncée intense alternait avec des bandes grisâtres dans les sections du cortex rénal. Nous avons pris -
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...
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EMT seob iPhone'i ostjad aastateks enda külge / Henrik Ilves
Ilves, Henrik
2008-01-01
EMT turundusjuhi Anne Muldme väitel hakatakse Eestis nii nagu Soomes ja Rootsiski iPhone'i puhul rakendama pakettide põhimõtet. iPhone'i tulevase hinnapoliitika põhimõtted Eestis on sarnased EMT omanikfirma TeliaSonera kehtestatuga Soomes ja Rootsis
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iPhone and iPad App 24-Hour Trainer
Mishra, Abhishek
2012-01-01
An all-in-one tutorial for planning, developing, and launching iPhone and iPad apps The number of applications in the Apple app store is growing at a staggering rate. Want to get in the game, but don't know iOS? This book-and-DVD package will help! With even little or no prior programming experience, you can learn the code necessary to build an app by following the how-to instructions in this book-and-video combo. Comprised of clear, no-nonsense lessons, the book walks you through each tutorial and then encourages you to work through simple exercises so that you can immediately apply what you
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Directory of Open Access Journals (Sweden)
Diazgranados Cristina
2007-06-01
Full Text Available
En el presente estudio se obtuvo un 46% de regeneración en explantes sin cocultivo de <i> i>Solanum phurejai> (Juzet <i>et> Buck variedad Yema de huevo clon 1 con la utilización del medio CIP-MS suplementado con reguladores de crecimiento (2 mg· L-1 ZR, 0,02 mg· L-1 ANA, 0,02 mg· L-1 AG3. A fin de mejorar los porcentajes de regeneración se modificó la proporción citoquininas:auxinas del CIP-MS obteniéndose un 55% de formación de callo con concentraciones de 2 mg· L -1 ZR y 0,04 mg· L-1 ANA. Posteriormente se evaluó el efecto de tres concentraciones de ácido giberélico (0,02 mg· L-1 , 0,05 mg· L-1 y 0,1 mg· L-1 obteniéndose un 87,6% de callogénesis y un 48,6% de regeneración mediante la utilización de un medio que contenía 2 mg· L-1 ZR, 0,04 mg· L-1 ANA, 0,02 mg· L-1 AG3 en explantes bajo condiciones de cocultivo. Estos resultados fueron corroborados en un segundo ensayo de regeneración bajo condiciones de cocultivo con <i>Agrobacterium tumefaciensi> cepa LBA4404 transformada mediante el vector binario pNOV022, de lo cual se obtuvieron porcentajes de callogénesis y regeneración muy similares. Previamente se reportó un promedio de 4 ± 3,3 regenerantes por callo utilizando entrenudos de <i>S. phurejai> mientras en este estudio se obtuvieron 15,2 ± 3 regenerantes por callo.
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The contribution of I. I. Tegako to anthropology of Belarus
Directory of Open Access Journals (Sweden)
Hurbo Tatyana
2016-01-01
Full Text Available L.I. Tegako started her studies of anthropology in the middle of 1960s when she received her research degree. The scope of her scientific interest included odontology and dermatoglyphics. For more than 40 years L.I. Tegako was a permanent supervisor of the group and later of the Anthropology department. From the beginning of 1970s Belarusian anthropologists started conducting complex anthropologic studies of the adult population of the republic in various parts of the country (anthropometry, anthroposcopy, dermatoglyphics, blood group factor. Comprehensive approach became the basis for studies of anthropogenic variety of local population on the territory of Belarus. In 1998 the team of anthropologists supervised by L.I. Tegako won a State Prize of the Republic of Belarus for the scope of work on the topic 'Person and his biocultural adaptation'. In 2000s the scope of L.I. Tegako's scientific interest included the determination of intersystemic correlations between dermatoglyphical and psychosomatic characteristics. During her academic career, L.I. Tegako published 17 monographs, 6 study guides, 11 brochures and 209 research papers. Lidiya Ivanovna worked at leading universities in the country; she had 7 students who completed PhD thesis. Since 1999 she worked as a professor of biology. L.I. Tegako deserves a credit for the organization of anthropological conferences in Minsk. She devoted a lot of attention to the international cooperation. As a result, L.I. Tegako contributed to the establishment of stable scientific and friendly ties with Serbian colleagues: Institute of History of the NAS of Belarus and Matica Srpska made a partnership agreement. This includes exchange of experience, scientific works, and realization of joint projects.
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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
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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
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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...
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DEFF Research Database (Denmark)
Roy, Krisna; Bertelsen, Mads Frost; Pors, Susanne Elisabeth
2014-01-01
p.i. (9.4 ± 1.4 g/l) and day 6 p.i. (8.0 ± 0.7 g/l) and the PT was prolonged at day 6 p.i. (168.1 ± 21.0 sec) compared with the day 0 standards (2.6 ± 0.2 g/l and 104.6 ± 2.0 sec, respectively) (P ... mean values of the clot formation rate (α-angle) and maximal amplitude (MA) of TEG tracing at day 3 p.i. (83.1 ± 0.7°, 83.8 ± 1.4 mm) and day 6 p.i. (84.0 ± 0.4°, 89.8 ± 1.0 mm) compared with the day 0 values (75.8 ± 2.2° and 66.9 ± 1.4 mm, respectively) (P ... (1.5 ± 0.1 g/l), PT (79.4 ± 6.4 sec), TEG-α (76.7 ± 1.5°) and TEG-MA (64.0 ± 2.3 mm) were lower at day 6 compared with values observed for the infected chickens (P
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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...
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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...
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Synthesis and biologic studies of iodinated (125I/127I) ethidium
International Nuclear Information System (INIS)
Ho Nanhui; Tumeh, Paul C.; Kassis, Amin I.
2001-01-01
An iodinated ( 125 I/ 127 I) ethidium derivative (3,8-diamino-5-[6'-(p-iodobenzoylamino)-4'-azahexyl]-6 -phenylphenanthridinium chloride hydrochloride) was synthesized and characterized. The labeling yield of the 125 I-labeled derivative was 75% for carrier-free 125 I, with a radiochemical purity of 95%. The incubation of iodoethidium with calf thymus DNA resulted in a substantial enhancement of fluorescence yield, indicating the intercalation of this compound into DNA. In the presence of iodoethidium, the nuclei of methanol-treated mammalian cells fluoresced, while those of viable cells did not (since the plasma membrane is impermeable to iodoethidium). When viable cells were incubated with the reduced form of the derivative, 125 I/ 127 I-dihydroethidium traversed the plasma membrane, was oxidized in the cytoplasm, and intercalated into nuclear DNA. Finally, we tested the hypothesis that larger malignant solid tumors, containing a relatively greater percentage of degenerating permeable cells, can be targeted with 125 I-ethidium. In-vivo studies demonstrated a small but positive correlation (R = 0.72) between tumor volume and the uptake of the derivative. Because of the ubiquitous presence of abnormal permeable cells and necrosis in tumors, our results support the belief that radiolabeled DNA-intercalating or DNA-binding molecules may be of diagnostic and therapeutic value for a variety of solid tumors in humans
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Directory of Open Access Journals (Sweden)
Viñuela Suárez, Eduardo
2010-02-01
Full Text Available Adapting a novel into a film is not a simple question of translating the narrative plot from one medium to another, it also deals with the cultural competence of the reader / spectator and the meanings and discourses articulated in the interpretation of the texts. Music is present in both novels and films, and so it participates in the process of creating meanings, even when it does not take part in the formal structure of the text. This article develops the analysis of popular music in <i>The Commitmentsi> and <i>High Fidelityi>, two films based on two novels where the ideology of rock music influences both characters and spaces. Songs, musicians and instruments are present in prose, dialogues, sound and image, both in the novels by Roddy Doyle and Nick Hornby, respectively, and in the films directed by Alan Parker and Stephen Frears, thus demonstrating the multimedia condition of music and its capability to interact with the narrative structure of the texts in the articulation of several discourses.
La adaptación cinematográfica de novelas va más allá de la transposición de la narrativa de un medio de expresión a otro e implica a una serie de discursos que están presentes en la obra y que son activados por el lector-espectador en función de su competencia cultural. La música, presente en ambos medios, es capaz de vehicular mensajes que, aunque no se encuentren en la estructura narrativa de las obras, participan en la creación de significados. En este artículo abordamos un análisis formal y discursivo de la música popular en <i>Alta fidelidadi> y <i>The Commitmentsi>, dos novelas adaptadas al cine en las que la ideología de la cultura rock se manifiesta en personajes y espacios. Canciones, músicos e instrumentos aparecen en texto escrito, diálogos, imagen y sonido en las novelas de Roddy Doyle y Nick Hornby y sus respectivas adaptaciones cinematográficas de Alan Parker y Stephen Frears, demostrando la condici -
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....
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Directory of Open Access Journals (Sweden)
Aragón Sandra
2008-08-01
Full Text Available
Para la toma adecuada de decisiones en el manejo de la mosca blanca de los invernaderos, se han propuesto diferentes criterios que buscan reducir el impacto producido por el uso indiscriminado de insecticidas. Sin embargo, estos criterios de decisión son aplicables principalmente al control químico de plagas y no se conoce su uso en programas de control biológico. Por lo tanto, con este trabajo se propuso generar unos criterios de decisión para el control biológico de la mosca blanca de los invernaderos <i>T. vaporariorumi> con el parasitoide <i>Encarsia formosai>, teniendo en cuenta su respuesta funcional y porcentaje de control. En un diseño completamente al azar se ubicaron 18 plantas de tomate de 8 semanas de edad infestadas con 500 ninfas de mosca blanca y 3 plantas como control absoluto. En cada planta se liberaron 0, 10, 20, 30, 40 y 50 adultos de <i>E. formosai>, con tres repeticiones por densidad. Se registró el número de ninfas melanizadas por efectos del parasitismo de <i>E. formosai> a los 12; 19 y 26 días después de la primera liberación y, al finalizar la cosecha, se realizó un conteo de las ninfas de mosca blanca totales. Los resultados de este trabajo sugieren que para obtener un control cercano a 80% se debe liberar un parasitoide por cada 17 ninfas de mosca blanca de tercer instar, evaluando el control después de 35 días. Se pueden realizar nuevas liberaciones de E. formosa dirigidas al control de las nuevas ninfas encontradas en los muestreos periódicos del cultivo.
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Outdoor i undervisningen på pædagoguddannelserne i VIA
DEFF Research Database (Denmark)
Kjeldsen, Lis Reinholdt
2011-01-01
En undersøgelse af hvorvidt undervisere på pædagoguddannelserne i VIA anvender andre kontekster end det traditionelle undervisningslokale i deres undervisning. Der er fokus på omfang og valg af udeområder....
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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...
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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.
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Erhvervsøkonomi i Slagelse i 25 år
DEFF Research Database (Denmark)
Scheuer, Steen; Andersen, Torben; Hansen, Kristian Rune
2010-01-01
Denne rapport behandler tre problemområder: 1. Om og i hvilket omfang det er lykkedes at fastholde dimittenderne fra Campus Slagelse beskæftigelsesmæssigt i regionen.2. Hvor mange der har taget en uddannelse oven på HA- eller HD-graden, fx en cand. merc., en cand. merc. aud., eller en masteruddan...
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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....
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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....
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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...
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Effects of spin-orbit coupling on laser cooling of BeI and MgI
Energy Technology Data Exchange (ETDEWEB)
Wan, Mingjie, E-mail: wanmingjie1983@sina.com; Huang, Duohui; Shao, Juxiang; Li, Yuanyuan [Computational Physics Key Laboratory of Sichuan Province, Yibin University, Yibin 644007 (China); Yu, You [College of Optoelectronic Technology, Chengdu University of Information Technology, Chengdu 610225 (China); Li, Song [College of Physical Science and Technology, Yangtze University, Jingzhou 434023 (China)
2015-10-28
We present the ab initio study of spin-orbit coupling effects on laser cooling of BeI and MgI molecules. Potential energy curves for the X{sup 2}Σ{sup +}{sub 1/2}, A{sup 2}Π{sub 1/2,3/2}, and 2{sup 2}Π{sub 3/2,1/2} states are calculated using multi-reference configuration interaction method plus Davidson corrections. Spectroscopic parameters of BeI and MgI are in excellent agreement with available experimental and theoretical values. The A{sup 2}Π{sub 3/2} state of MgI is a repulsive state. It is an unsuitable scheme for the A{sup 2}Π{sub 3/2}(υ′)← X{sup 2}Σ{sup +}{sub 1/2} (υ″) transition for laser cooling of MgI. Highly diagonally distributed Franck-Condon factors f{sub 00} for the A{sup 2}Π{sub 1/2,3/2} (υ′ = 0) ← X{sup 2}Σ{sup +}{sub 1/2} (υ″ = 0) transitions and suitable radiative lifetimes τ for the A{sup 2}Π{sub 1/2,3/2} (υ′ = 0) of BeI and MgI are obtained. Three laser wavelength drives are required for the A{sup 2}Π{sub 1/2,3/2}(υ′)←X{sup 2}Σ{sup +}{sub 1/2} (υ″) transitions of BeI and MgI. The proposed cooling wavelengths of BeI and MgI are both in the violet region. The results imply the feasibility of laser cooling of BeI and MgI, and that laser cooling of BeI is more possible.
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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....
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iSpike: a spiking neural interface for the iCub robot
International Nuclear Information System (INIS)
Gamez, D; Fidjeland, A K; Lazdins, E
2012-01-01
This paper presents iSpike: a C++ library that interfaces between spiking neural network simulators and the iCub humanoid robot. It uses a biologically inspired approach to convert the robot’s sensory information into spikes that are passed to the neural network simulator, and it decodes output spikes from the network into motor signals that are sent to control the robot. Applications of iSpike range from embodied models of the brain to the development of intelligent robots using biologically inspired spiking neural networks. iSpike is an open source library that is available for free download under the terms of the GPL. (paper)
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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.
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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...
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Klimadiplomatiets afveje i Kyoto-processen
DEFF Research Database (Denmark)
Czeskleba-Dupont, Rolf
2014-01-01
Operationaliseringen af de i 1992 i Rio indgåede forpligtelser i forhold til UNFCCC (FNs Rammekonventionom klimaforandringerne) blev siden COP3 i Kyoto under dominerende indflydelse af USAs forhandlere. Kritik af resultaterne kom allerede i 1998 fra den tyske regerings rådgivere i WGBU, men blev...
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Kontraktstyring i den kommunale sagsbehandling
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
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Indian Academy of Sciences (India)
letter, I arrived in Calcutta two days early; in order not to alarm you, however, I wanted to wait for the ... It is strange to know that from my window I could see the gigantic ... I have witnessed some things which I would rather wish I could forget.
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Kevin, Li Xinpeng
2012-01-01
The goal of this exploratory research is to identify attributes that can distinctly characterize iPad and examine the predictive power of iPad attributes, lifestyles, media dependency, and demographics on adoption of iPad, iPad usage patterns and intensity of iPad usage. Using a snowballing sample, an online survey was conducted with 623 university students in Mainland China, among which 217 were iPad users and 406 were non-users. Results of regression analyses show that application affordanc...
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Primary dispersal of <i>Cytisus multiflorusi> seeds
Directory of Open Access Journals (Sweden)
Moreno Marcos, G.
1992-12-01
Full Text Available Current work relates to the primary dispersal of <i>Cytisus multiflorusi> seeds: seed production, dispersal mechanisms, distribution of seed after dissemination and dormancy were investigated. The distribution of seed was recorded before collection; seed viabilaty and dormancy were assessed. The results indicate that the dispersion is assisted by an explosive dehiscence of the seed pod in that only 35% of the seed drop directly beneath the plant, the remainder falling further away, some reaching distances greater than 3 m. Dormancy index was found to be 98.9%.
[es] En el presente trabajo se estudia la dispersión primaria de las semillas de <i>Cytisus multiflorusi>: cuantificación de la producción, mecanismos de dispersión, distribución de las semillas tras la diseminación, y su estado de latencia. Para ello se colocaron dispositivos de recogida de las semillas debajo y alrededor de las plantas, anotando la cantidad y distancia de la calda; posteriormente eran sembradas para comprobarla viabilidad y dormición de las semillas. Los resultados indican la existencia de una dispersión por dehiscencia explosiva; solamente el 35% de las semillas caen bajo la planta, haciéndolo el resto en sus alrededores; algunas alcanzaron distancias superiores a los 3 m. El índice de dormición es de 98.9%.
[fr] Dans le présent travail on étudie la dispersion primaire des graines de <i>Cytisus multiflorusi>: quantification de la production, mécanismes de dispersion, distribution des graines après la dissémination et leur état de latence. Pour ce faire on a placé des dispositifs de récolte des graines au dessous et autour des plantes, enregistrant la quantité et la distance de la chute; après elles étaient semées pour tester la viabilité et dormance des graines. Les résultats indiquent l'existence d'une dispersion par déhiscence explosive; seulement 35% des graines tombent au-dessous de la plante, et le reste dans les -
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...
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Efekti i mehanizmi delovanja interleukina-17 na funkcije mezenhimskih i endotelskih ćelija
Kocić, Jelena S.
2012-01-01
Interleukin (IL) 17 familija citokina se smatra najmanje istraženom citokinskom familijom sa značajnom ulogom u procesu inflamacije. Ubikvitarna rasprostranjenost receptora za IL-17 omogućava ovom citokinu mnogobrojne biološke efekte u organizmu, poput važne uloge u imunskom odgovoru i regulaciji procesa hematopoeze, ali i u progresiji različitih bolesti, uključujući inflamatorna, autoimunska i maligna oboljenja. IL-17 ima ulogu u ćelijskim procesima poput proliferacije i difer...
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SOCIJALNA KOMPETENCIJA I ZVANJE RAVNATELJA OSNOVNIH I SREDNJIH ŠKOLA
Janković, Marija
2012-01-01
Ravnatelj svojim djelovanjem (bez obzira ovisi li o situaciji ili ciljevima) ne utječe samo na konačan uspjeh škole, nego i na međuljudske odnose, na školsku klimu, na motivaciju nastavnika i organizaciju rada škole. Upravo iz tog razloga nužno je ispitati socijalnu kompetenciju ravnatelja kako bi se potaknulo njihovo osposobljavanje, a potom i njihovo usavršavanje, jer vježbom se socijalne vještine mogu usavršiti (Goleman, 2008). Cilj rada je ispitati socijalne vještine koje su pr...
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Flight Deck I-Glasses, Phase I
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...
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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
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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...
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International Nuclear Information System (INIS)
Kulbachinskii, Vladimir A.; Kytin, Vladimir G.; Kudryashov, Alexey A.; Kuznetsov, Alexei N.; Shevelkov, Andrei V.
2012-01-01
The electronic structures were calculated for BiTeBr and BiTeI using the density-functional theory approach and accounting for the strong spin–orbital interaction. Qualitatively, the band structures for two compounds are similar, showing strong mixing of the p states of all elements in vicinity of the Fermi level, with the band gaps of 0.595 and 0.478 eV for BiTeBr and BiTeI, respectively. The optimized crystal structures show a tendency for the Bi–X (X=Br, I) bond elongation compared to the Bi–Te one. Both compounds are intrinsic n-type semiconductors but display a metallic-like conductivity coupled to rather large thermopower, which is rationalized within the frames of the acoustic phonons scattering model. Because of larger thermopower BiTeBr exhibits a twice higher thermoelectric figure-of-merit near room temperature, ZT=0.17, compared to BiTeI. The addition of 1 mass% of BiI 3 or CuI to BiTeI decreases the mobility of electrons by two orders of magnitude, leading to significantly lower electrical conductivity, but at the same time effectively reduces the thermal conductivity. The prospects of further enhancing the thermoelectric efficiency are briefly discussed. - Graphical abstract: View of the crystal structure of BiTeBr is shown in the figure The optimized crystal structures show a tendency for the Bi–X (X=Br, I) bond elongation compared to the Bi–Te one. The electronic structures were calculated for BiTeBr and BiTeI using the density-functional theory approach and accounting for the strong spin–orbital interaction. Qualitatively, the band structures for two compounds are similar, showing strong mixing of the p states of all elements in vicinity of the Fermi level, with the band gaps of 0.595 and 0.478 eV for BiTeBr and BiTeI, respectively. Both compounds are intrinsic n-type semiconductors but display a metallic-like conductivity coupled to rather large thermopower, which is rationalized within the frames of the acoustic phonons scattering
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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...
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DEFF Research Database (Denmark)
Trondhjem, Naja Blytmann
. Verbalstammerne har en inhærent valensangivelse og kan opdeles i: a) altid intransitive som tilføjes intransitiv fleksion knytter et subjekt i absolutiv kasus, b) altid transitive, som tilføjes den transitive fleksion knytter subjekt i relativ kasus og objekt i absolutiv kasus og c) ’ambitransitive’ (ifl. Mithun...... (2000)), som enten kan tilføjes intransitive eller transitive fleksionsbøjning afhængigt af fokus på argumentet. De altid transitive kan yderligere inddeles i divalente og trivalente verber. De trivalente verber knytter subjekt i relativ, direkte objekt i absolutiv og det tredje argument i oblik kasus...... ex. indirekte objekt i allativ kasus. De ambitransitive er ofte divalente verbalstammer. De ambitransitive verber kan reducere valens ved at omdanne en aktiv transitiv sætning til en intransitiv antipassiv sætning ex. angutip neqi nerivaa ’manden spiser kødet’, (subjekt i relativ og objekt i...
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Bygningsgennemgang som led i energistyring i større bygninger
DEFF Research Database (Denmark)
Dinesen, J.; Mørk, P.
Rapporten indeholder materiale til brug ved planlægning og udførelse af en detaljeret bygningsgennemgang i forbindelse med etablering af løbende energistyring. Rapporten udbygger SBI-rapport 117: Et system for energiøkonomisk drift i større bygninger....
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131I Metaiodobenzylguanidine (131I MIBG) kinetics in a carcinoid tumor
International Nuclear Information System (INIS)
Schiavo, R.; Concolino, G.; Fazi, F.; Iannantuono, P.; Voti, S. Li; Manzara, A.; Pavoni, P.
1987-01-01
The 131 I-MIBG kinetics was studied in vivo in patients with carcinoid tumors and liver metastases. Activity curve analysis showed that the maximum uptake of 131 I-MIBG in a carcinoid tumor occurred after 48 hours, while its biological half time was of 8 days and a half. Although more data are necessary to understand a significant variation in 131 I-MIBG kinetics between the different kinds of APUD neoplasms, it is thought that a dynamic-funtional study allowing the evaluation of the different biological half-time, could be helpful for the selection of these neoplasms, which could be treated with 131 I-MIBG. Radiation doses required for the treatment are also estimated. (M.E.L.) [es
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Kloakering i landbruget og ved mindre beboelser i landzone
DEFF Research Database (Denmark)
Petersen, H.W.
Publikationen er beregnet til at bruges sammen med DIF's "Norm for afløbsinstallationer", DS 432, og "Norm for mindre afløbsanlæg med nedsivning", DS 440. Den giver vejledning i planlægning og udførelse af afløbsanlæg med rørledninger i jord for ajle og spildevand ført til opbevaringstank eller v...
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Jernkarbonatutfelling (Sideritt) i myr
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...
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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....
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The strategy for advanced I and C system (I3CS) development
International Nuclear Information System (INIS)
Kim, J.T.; Ham, C.S.; Kwon, K.C.; Lee, D.Y.
1995-01-01
All of the nuclear power plants in Korea are operating with analog instrumentation and control (I and C) equipment that are increasingly faced with frequent troubles, obsolescence and high maintenance expense. Electrical and computer technology has improved rapidly in recent years and has been applied to other industries. So it is strongly recommended we adopt modern digital and computer technology to improve plant safety and availability. We established the plan for the development of advanced I and C technology and equipment to solve the above problems. This plan is aimed at replacement of the I and C systems for the existing plants, and at planned as well as next generation nuclear power plants. It is divided into three major parts as follows: Plan for domestic design of I and C systems and components; plan for domestic manufacturing of I and C equipment; plan for development of future technologies. According to the above strategy, the advanced I and C system, namely, I 3 CS(Integrated Intelligent Instrumentation and Control System) will be developed for after next generation NPPs. I 3 CS Consist of three major parts, the advanced compact workstation, distributed digital control and protection system, and the computer-based alarm processing and operator support system, namely, DREAMS (Diagnosis, Response, and operator Aid Management System). The first stage for DREAMS is to develop DAS (Dynamic Alarm System) which reduces the nuisance alarm based on operating mode, direct precursor, and dynamic prioritization. (author). 6 refs, 2 figs
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The Bluedisks project, a study of unusually H I-rich galaxies - I. H I sizes and morphology
Wang, Jing; Kauffmann, Guinevere; Jozsa, Gyula I. G.; Serra, Paolo; van der Hulst, Thijs; Bigiel, Frank; Brinchmann, Jarle; Verheijen, M. A. W.; Oosterloo, Tom; Wang, Enci; Li, Cheng; den Heijer, Milan; Kerp, Juergen
2013-01-01
We introduce the 'Bluedisk' project, a large programme at the Westerbork Synthesis Radio Telescope that has mapped the H i in a sample of 23 nearby galaxies with unusually high H i mass fractions, along with a similar-sized sample of control galaxies. This paper presents the sample selection,
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”Å skape forbindelser”. Atelierets plass og atelieristens kompetanse i barnehagene i Reggio Emilia
Directory of Open Access Journals (Sweden)
Kari Carlsen
2013-06-01
Full Text Available Forming har gitt viktige bidrag til det helhetlige kunnskapssyn som lenge har vært dominerende i norsk barnehagepedagogikk, men fagfeltet er nå lavere prioritert i forhold til aktiviteter som er ment å fremme ferdigheter innenfor språk og matematikk. Debatten om kvalitet i norsk barnehage proble-matiserer krav om testing av små barns ferdigheter innenfor avgrensede kunnskapsområder. Med et formingsfaglig fokus er det av interesse å se nærmere på barnehagene i Reggio Emilia, Italia, der barns praktisk skapende virksomhet gis høy prioritet med et atelier i hver barnehage. Den anerkjente kvaliteten i disse barnehagene (Wingert & Kantrowitz, 1991; Gardner, 2001; Gardner, 2006 begrun-ner en nærmere undersøkelse av atelierets betydning i denne pedagogiske praksisen.Målsettingen med artikkelen er å belyse atelierets funksjon i barnehagene i Reggio Emilia, hvilken rolle estetisk skapende virksomhet oppfattes å ha i barns læring, og å undersøke den kompetansen som kreves av atelieristen med ansvar for arbeidet i atelieret. Artikkelens empiriske materiale er hentet inn gjennom et intervju med atelierist Vea Vecchi i april 2010.Med en fenomenologisk-hermeneutisk tilnærming drøftes metodiske spørsmål omkring kvalitativt intervju og behandling av kvalitative data ved hjelp av oversetter. Metodiske valg i forbindelse med intervjuet som er gjennomført diskuteres. Resultater av intervjuet presenteres og analyseres, og atelierets egenart og atelieristens kompetanse diskuteres. Begrepet atelierismen etableres. Katego-riene som er resultat av analysen synes brukbare som utgangspunkt for undersøkelser av atelierismen i ulike kontekster.Søkeord: Atelier, atelierist/atelierista, atelierismen, estetiske læreprosesser, kvalitativt intervju, Reggio Emilia.
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Thermoactivation processes in PbI2:Zr and PbI2 crystals
International Nuclear Information System (INIS)
Panasyuk, M.R.; Kapustyanik, V.B.; Tsibul's'kij, V.S.; Dubov, Yu.G.; Pasternak, R.M.
2007-01-01
The X-ray luminescence, thermal emission and thermally stimulated depolarisation spectra as well as the influence of IR-illumination on the thermal emission and thermally stimulated depolarisation spectra of the PbI 2 :Zr and PbI 2 crystals have been studied. There were found the hole traps in the PbI 2 :Zr crystals that are absent in PbI 2 . For the observed traps the activation energy has been calculated. The mechanisms describing the traps' nature and that of thermally stimulated depolarisation currents have been proposed
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Directory of Open Access Journals (Sweden)
Anna Mokrowiecka
2010-12-01
Full Text Available Nudności są nieprzyjemnym, niebolesnym odczuciem silnej, nieodpartej potrzeby zwymiotowania, z kolei wymioty polegają na gwałtownym wydaleniu treści żołądka przez usta w wyniku silnych skurczów mięśni brzucha i klatki piersiowej. Nudności i wymioty mogą występować niezależnie od siebie, ale najczęściej są ze sobą powiązane. Nudności i wymioty powstają w odpowiedzi zarówno na bodźce fizjologiczne, jak i patologiczne. Zwykle jednak przewlekłe nudności i wymioty są objawem chorób przewodu pokarmowego lub innych narządów. Choroby przewodu pokarmowego i otrzewnej stanowią szeroką grupę chorób wywołujących nudności i wymioty, począwszy od nieżytu żołądkowo-jelitowego o ostrym infekcyjnym tle, przez zatrucie pokarmowe i nadwrażliwość pokarmową, do stanów ostrych – niedrożności jelita cienkiego lub/i grubego. Leki i toksyny, między innymi cytostatyki, są jedną z najczęstszych przyczyn nudności i wymiotów. Wśród innych przyczyn nudności i wymiotów można wymienić: choroby OUN prowadzące do zwiększenia ciśnienia śródczaszkowego, choroby endokrynologiczne i metaboliczne (mocznica, kwasica ketonowa w cukrzycy, choroby tarczycy i przytarczyc oraz choroba Addisona, zawał ściany dolnej mięśnia sercowego, nudności i wymioty pooperacyjne. Nudności i wymioty jako choroby czynnościowe zostały ujęte w III kryteriach rzymskich w punkcie „Choroby czynnościowe żołądka i dwunastnicy”. W diagnostyce różnicowej należy uwzględnić czas trwania wymiotów, czas pomiędzy posiłkiem a wystąpieniem wymiotów, a także charakter treści wymiocin i inne objawy towarzyszące. Wywiad i badanie fizykalne dostarczają zwykle wystarczających informacji do postawienia prawidłowej diagnozy i ustalenia leczenia u większości chorych cierpiących na nudności i wymioty. Niektórzy pacjenci wymagają jednak badań laboratoryjnych, obrazowych i endoskopowych przed zastosowaniem odpowiedniej
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International Nuclear Information System (INIS)
Lagunas-Solar, M.C.
1985-01-01
The use of iodine-123 in nuclear medicine procedures is well documented in the scientific literature. Also, several methods for its production based on accelerator techniques have been described. Indirectly made 123 I via the 127 I(p,5n) 123 Xe → 123 I reaction produces 123 I of > 99.9% radionuclidic purity, with only 125 I ( 123 I production were developed at the University of California at Davis, where since 1974 the 76-in. isochronous cyclotron of the Crocker Nuclear Laboratory has been used for routine biweekly production of high-purity no-carrier-added 123 I
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Cooper, R; Blashfield, R K
2016-02-01
The DSM-I is currently viewed as a psychoanalytic classification, and therefore unimportant. There are four reasons to challenge the belief that DSM-I was a psychoanalytic system. First, psychoanalysts were a minority on the committee that created DSM-I. Second, psychoanalysts of the time did not use DSM-I. Third, DSM-I was as infused with Kraepelinian concepts as it was with psychoanalytic concepts. Fourth, contemporary writers who commented on DSM-I did not perceive it as psychoanalytic. The first edition of the DSM arose from a blending of concepts from the Statistical Manual for the Use of Hospitals of Mental Diseases, the military psychiatric classifications developed during World War II, and the International Classification of Diseases (6th edition). As a consensual, clinically oriented classification, DSM-I was popular, leading to 20 printings and international recognition. From the perspective inherent in this paper, the continuities between classifications from the first half of the 20th century and the systems developed in the second half (e.g. DSM-III to DSM-5) become more visible.
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Pedro de Valencia, Francisco de Gurmendi and the <i>Plomos de Granadai>
Directory of Open Access Journals (Sweden)
Magnier, Grace
2003-12-01
Full Text Available The Inquisition in 1618 seized all the papers of Pedro de Valencia, renowned humanist, biblical scholar and chronicler of Philip III. The papers of other members of his circle were also confiscated. In this article I examine the two most important texts: <i>Sobre el pergamino y láminas de Granadai> of Valencia and a <i>Libelo Segundoi> of Francisco de Gurmendi, interpreter of Oriental languages for Philip III. The article is structured around Gurmendi's response to a memorial published in 1617 by Archbishop Pedro de Castro in defence of one of his translators. Gurmendi, working from his translation of the first two Lead Books, shows how these contain heretical ideas on the Trinity and how the <i>memorialista> has mistranslated many passages.
En 1618 la Inquisición confiscó todos los papeles de Pedro de Valencia, humanista insigne, exegeta bíblico y cronista del reino y de las Indias de Felipe III. También confiscó los papeles de otros miembros de su círculo. En este artículo examino los dos textos más importantes: <i>Sobre el pergamino y láminas de Granadai> de Valencia y un <i>Libelo segundoi> de Francisco de Gurmendi, intérprete en lenguas orientales del rey. El artículo se centra en la respuesta de Gurmendi a un memorial del Arzobispo Pedro de Castro en el que se defiende a uno de sus traductores. Partiendo de su traducción de los dos primeros libros plúmbeos, Gurmendi muestra las herejías trinitarias que éstos contienen y cómo el memorialista ha traducido mal muchos pasajes.
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Panel: If I Only Knew Then What I Know Now
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
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<i>Shurṭa> Chiefs in Basra in the Umayyad Period: a Prosopographical Study
Directory of Open Access Journals (Sweden)
Ebstein, Michael
2010-06-01
Full Text Available The article describes some of the functions and characteristics of the <i>shurṭa> in the Umayyad era, through an analysis of the men who served as <i>shurṭa> chiefs in Basra during this period. The <i>shurṭa>, as is shown in the article, was an important body which helped the Umayyad regime and its regional governors in Iraq and Basra to consolidate the Umayyad governmental system. The <i>shurṭa> offered the governor of Baṣra personal protection and was at the same time a symbol of his authority and power. It was responsible for the maintenance of public order and security in Basra and dealt with individuals who transgressed common religious norms. Another important duty performed by the shurta in Basra and nearby areas was to fight against different groups of <i>khawārij> as well as various other rebels and oppositional forces. In the article, attention is drawn to political and social issues such as the <i>ashrāf> and their traits; intertribal conflicts; marital ties, etc. By discussing these different subjects, the article aims to reintroduce an often ignored method into the research field of early Islamic history — prosopography.
El artículo describe algunas de las funciones y características de la šurta durante la época omeya, por medio de un análisis de los hombres que sirvieron como jefes de la misma en Basora durante este período. La šurta, tal y como se explica en el artículo, era una institución importante que ayudaba al régimen omeya y sus gobernadores regionales en Iraq y Basora, en su tarea de consolidar el sistema gubernamental omeya. La <i>šurta> ofrecía protección personal al gobernador y era, a su vez, un símbolo de su autoridad y poder; era responsable del mantenimiento del orden público y la seguridad y se ocupaba de los individuos que transgredían las normas religiosas comunes. Otra tarea importante de la <i>šurta> en Basora y zonas cercanas era la de luchar contra -
Chambers, Mark L
2012-01-01
The bestselling guide to the ultimate all-in-one computer—now updated and revised throughout! If you're looking for speed, performance, and power, the iMac is the ultimate all-in-one computer. From its superior performance, powerful operating system, and amazing applications, the iMac is one awesome machine, and the fun, friendly, and approachable style of iMac For Dummies is an ideal way to get started with the basics. You'll learn the fundamentals of the iMac including setting up and customizing your iMac and the software that comes with it, importing files from your old computer, send
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McFedries, Paul
2010-01-01
Everything everyone wants to know about using the Apple iPad. On January 27, 2010, Apple announced the latest in its line of revolutionary, ultraportable devices - the iPad. iPad Portable Genius is the latest in a line of ultra handy, go-to and goes-with you anywhere guides for getting the most out of a new Apple product. Written to provide readers with highly useful information that's easily accessible, iPad Portable Genius is full of tips, tricks and techniques for maximizing each of the iPad's most popular features.:; Designed in full-color with an Apple look and feel, and written in a hip,
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International Nuclear Information System (INIS)
Quirion, R.; Rowe, W.; Kar, S.; Dore, S.
1997-01-01
The insulin-like growth factors (IGF-I and IGF-II) and insulin are localized within distinct brain regions and their respective functions are mediated by specific membrane receptors. High densities of binding sites for these growth factors are discretely and differentially distributed throughout the brain, with prominent levels localized to the hippocampal formation. IGFs and insulin, in addition to their growth promoting actions, are considered to play important roles in the development and maintenance of normal cell functions throughout life. We compared the anatomical distribution and levels of IGF and insulin receptors in young (five month) and aged (25 month) memory-impaired and memory-unimpaired male Long-Evans rats as determined in the Morris water maze task in order to determine if alterations in IGF and insulin activity may be related to the emergence of cognitive deficits in the aged memory-impaired rat. In the hippocampus, [ 125 I]IGF-I receptors are concentrated primarily in the dentate gyrus (DG) and the CA3 sub-field while high amounts of [ 125 I]IGF-II binding sites are localized to the pyramidal cell layer, and the granular cell layer of the DG. [ 125 I]insulin binding sites are mostly found in the molecular layer of the DG and the CA1 sub-field. No significant differences were found in [ 125 I]IGF-I, [ 125 I]IGF-II or [ 125 I]insulin binding levels in any regions or laminae of the hippocampus of young vs aged rats, and deficits in cognitive performance did not relate to altered levels of these receptors in aged memory-impaired vs aged memory-unimpaired rats. Other regions, including various cortical areas, were also examined and failed to reveal any significant differences between the three groups studied.It thus appears that IGF-I, IGF-II and insulin receptor sites are not markedly altered during the normal ageing process in the Long-Evans rat, in spite of significant learning deficits in a sub-group (memory-impaired) of aged animals. Hence
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DEFF Research Database (Denmark)
Pedersen, Ulla; Schulz, Anette
Dette materiale henvender til frivillige og ledere i foreninger samt til alle ildsjæle, der ønsker at bidrage til øget sundhed og udvikling i lokalområdet. Materialet er et resultat af udviklingsprojektet ”Landsbyer i bevægelse”, gennemført i 2007/2008 med støtte fra LEADER+ samt Landdistriktspul......Dette materiale henvender til frivillige og ledere i foreninger samt til alle ildsjæle, der ønsker at bidrage til øget sundhed og udvikling i lokalområdet. Materialet er et resultat af udviklingsprojektet ”Landsbyer i bevægelse”, gennemført i 2007/2008 med støtte fra LEADER+ samt...... Landdistriktspuljen v. Indenrigsministeriet og er tænkt som inspiration til igangsættelse af sundhedsfremmende udviklingsinitiativer i lokalområderne. Materialet indeholder en præsentation af projektet; en beskrivelse af de lokale udviklingsinitiativer, der blev igangsat i løbet af projektperioden; en præsentation af...
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Dynamics of the F(-) + CH3I → HF + CH2I(-) Proton Transfer Reaction.
Zhang, Jiaxu; Xie, Jing; Hase, William L
2015-12-17
Direct chemical dynamics simulations, at collision energies Erel of 0.32 and 1.53 eV, were performed to obtain an atomistic understanding of the F(-) + CH3I reaction dynamics. There is only the F(-) + CH3I → CH3F + I(-) bimolecular nucleophilic substitution SN2 product channel at 0.32 eV. Increasing Erel to 1.53 eV opens the endothermic F(-) + CH3I → HF + CH2I(-) proton transfer reaction, which is less competitive than the SN2 reaction. The simulations reveal proton transfer occurs by two direct atomic-level mechanisms, rebound and stripping, and indirect mechanisms, involving formation of the F(-)···HCH2I complex and the roundabout. For the indirect trajectories all of the CH2I(-) is formed with zero-point energy (ZPE), while for the direct trajectories 50% form CH2I(-) without ZPE. Without a ZPE constraint for CH2I(-), the reaction cross sections for the rebound, stripping, and indirect mechanisms are 0.2 ± 0.1, 1.2 ± 0.4, and 0.7 ± 0.2 Å(2), respectively. Discarding trajectories that do not form CH2I(-) with ZPE reduces the rebound and stripping cross sections to 0.1 ± 0.1 and 0.7 ± 0.5 Å(2). The HF product is formed rotationally and vibrationally unexcited. The average value of J is 2.6 and with histogram binning n = 0. CH2I(-) is formed rotationally excited. The partitioning between CH2I(-) vibration and HF + CH2I(-) relative translation energy depends on the treatment of CH2I(-) ZPE. Without a CH2I(-) ZPE constraint the energy partitioning is primarily to relative translation with little CH2I(-) vibration. With a ZPE constraint, energy partitioning to CH2I(-) rotation, CH2I(-) vibration, and relative translation are statistically the same. The overall F(-) + CH3I rate constant at Erel of both 0.32 and 1.53 eV is in good agreement with experiment and negligibly affected by the treatment of CH2I(-) ZPE, since the SN2 reaction is the major contributor to the total reaction rate constant. The potential energy surface and reaction dynamics for F
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Probiotyki i prebiotyki w profilaktyce i leczeniu chorób u dzieci
Directory of Open Access Journals (Sweden)
Leokadia Bąk-Romaniszyn
2010-11-01
Full Text Available Probiotyki to mikroorganizmy, które wywierają korzystne działanie na organizm gospodarza po spożyciu przez niego odpowiedniej dawki określonego szczepu. Najczęściej jako probiotyki stosowane są bakterie kwasu mlekowego Lactobacillus i Bifidobacterium oraz wybrane szczepy Streptococcus, Bacillus, a także drożdże Saccharomyces boulardii. Probiotyki wpływają korzystnie na skład środowiska ekologicznego przewodu pokarmowego, wykazując antagonizm w stosunku do drobnoustrojów chorobotwórczych kolonizujących błonę śluzową przewodu pokarmowego. Obecność w jelicie bakterii fermentacyjnych, głównie pałeczek kwasu mlekowego, a zwłaszcza typów adhezyjnych, chroni przed dyslokacją bakteryjną i enterotoksemią, normalizuje zaburzenia motoryki jelit i zmniejsza objawy nietolerancji laktozy. Prebiotyki to substancje naturalnie zawarte w żywności (bądź do niej dodawane, które selektywnie pobudzają wzrost i/lub aktywność wybranych szczepów bakterii probiotycznych obecnych w przewodzie pokarmowym. Probiotyki i prebiotyki, jak również ich połączenie – synbiotyki, występują w pożywieniu, mieszankach mlecznych, preparatach farmakologicznych, dodatkach do żywności, suplementach dietetycznych i w sposób naturalny poprawiają stan naszego zdrowia oraz mają coraz większe znaczenie w nowoczesnej medycynie. W pracy omówiono udział probiotyków i prebiotyków w kształtowaniu się biocenozy przewodu pokarmowego oraz w profilaktyce i leczeniu wybranych chorób u dzieci, jak: biegunki bakteryjne i wirusowe, dysbioza poantybiotykowa, martwica jelit noworodków, kolka jelitowa, nieswoiste zapalenia jelit, zaburzenia czynnościowe przewodu pokarmowego.
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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....
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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...
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2010-01-01
... 5 Administrative Personnel 2 2010-01-01 2010-01-01 false Can I cancel my FERS election if I was in the wrong retirement plan at the time I elected FERS coverage and I have an election opportunity under... ERRONEOUS RETIREMENT COVERAGE CORRECTIONS ACT Making an Election Fers Elections § 839.621 Can I cancel my...
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~ i t i d e r m a t o ~ h ~ t i c Activities of Nine (9) Essential Oils J.R. ...
African Journals Online (AJOL)
i t i d e r m a t o ~ h ~ t i c Activities of Nine (9) Essential Oils. J.R. KUIATE", S.P. KUATE'.~, N.E. KEMADJOU~, S. DJOKOUA~, F. ZIFACK~ AND J. ~. 0. ~. 0. ~. I Department of Biochemistry, FS, University of Dschang, P.O. Box 67 Dschang, Cameroon. 2~epartment of Biochemistry, FS, University of Yaoundt!, P.O. Box 812 ...
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Study of {sup 124} I contamination in {sup 123} I used in medical applications
Energy Technology Data Exchange (ETDEWEB)
El-Samman, H [Faculty of Science, Menoufia University, Shibin El-Kom (Egypt); Arafa, W [Physics Department, Faculty of Women, Ain Shams University, Cairo (Egypt)
1997-12-31
The decay of 0.2 mCi capsules of iodide ({sup 123} I) used for diagnostic purpose and delivered to hamad hospital in Qatar, was studied using HPGe detector of (30% efficiency and 1.8 KeV energy resolution), coupled to a computer based 4096 multichannel analyzer. The acquisition parameters were controlled by computer program. The gamma spectra were analyzed using well developed gamma spectrum analysis program gamanl. Results showed that the isotope used is not pure {sup 123} I but it is a mixture of {sup 123} I and {sup 124} I. The percentage of the unwanted {sup 124} I isotope was estimated to be 15%. The dose taken by the patient due to the unwanted {sup 124} I isotope was estimated. Half-lives time of the {sup 123} I and {sup 123} I isotopes were determined with high accuracy and compared to the published values. 3 tabs.
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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.)
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Mapping of epitopes on Poa p I and Lol p I allergens with monoclonal antibodies.
Lin, Z W; Ekramoddoullah, A K; Jaggi, K S; Dzuba-Fischer, J; Rector, E; Kisil, F T
1990-01-01
Allergen Poa p I isolated from the dialysed aqueous extract of Kentucky blue grass pollen by affinity chromatography with an anti-Lol p I murine monoclonal antibody (MAb) 290A-167 was previously shown to consist of a 35.8-kilodalton (kD) component with a pI of 6.4, designated as Poa p Ia, and a 33-kD component with a pI of 9.1, designated as Poa p Ib. The present study reports on the comparative antigenic analyses of these two components, using MAbs produced separately against Poa p I and Lol p I. Thus, anti-Poa p I MAbs 60 and 61 and anti-Lol p I MAb 290A-167 recognized Poa p Ia and Poa p Ib whereas anti-Poa p I MAbs 62, 63 and 64 and anti-Lol p I MAb 348A-6 recognized only Poa p Ia. The specificities of the MAbs were further resolved by comparing their respective abilities to inhibit the binding of 125I-Poa p I or 125I-Lol p I to the different MAbs prepared in the form of solid phase. These studies revealed that at least 4 distinct epitopes (designated as E1, E2, E3 and E4) were shared by both Poa p I and Lol p I. All 4 epitopes were present on Poa p Ia whereas only E1 and E3 were detected on Poa p Ib. E1 was recognized by MAbs 60 and 61, E2 by MAbs 62, 63 and 64, E3 by MAb 290A-167 and E4 by MAb 348A-6.(ABSTRACT TRUNCATED AT 250 WORDS)
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I think in portuguese I think in portuguese
Directory of Open Access Journals (Sweden)
Signe Oksefjell Ebeling
2008-10-01
Full Text Available This paper takes Aijmer (1997, 1998 and Simon-Vandenbergen’s (1998 contrastive work on I think as its starting point. In their studies, both Aijmer and Simon-Vandenbergen show that English think is a fuzzy verb and that this becomes particularly evident in a cross-linguistic perspective. Neither Swedish, Dutch or French seems to have one verb corresponding to the whole semantic range of think. In this article, the polysemous nature of think will be further explored in an English-Portuguese contrastive perspective. The data for the study will be taken from the English- Portuguese parallel corpus COMPARA. Portuguese equivalents of (I think will be analysed and the results compared to the findings of Aijmer and Simon-Vandenbergen. This paper takes Aijmer (1997, 1998 and Simon-Vandenbergen’s (1998 contrastive work on I think as its starting point. In their studies, both Aijmer and Simon-Vandenbergen show that English think is a fuzzy verb and that this becomes particularly evident in a cross-linguistic perspective. Neither Swedish, Dutch or French seems to have one verb corresponding to the whole semantic range of think. In this article, the polysemous nature of think will be further explored in an English-Portuguese contrastive perspective. The data for the study will be taken from the English- Portuguese parallel corpus COMPARA. Portuguese equivalents of (I think will be analysed and the results compared to the findings of Aijmer and Simon-Vandenbergen.
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Monoclonal antibodies directed to human insulin-like growth factor I (IGF I)
International Nuclear Information System (INIS)
Laubli, U.K.; Baier, W.; Celio, M.R.; Binz, H.; Humbel, R.E.
1982-01-01
Mouse hybridomas secreting antibodies to human insulin-like growth factor I (IGF I) were produced by fusion of spleen cells of hyperimmunised mice with FO mouse-myeloma cells. Eight clones producing antibodies against human IGF I have been isolated, two of which have been characterised. One was used in a radioimmunoassay, the other for immunopurification of IGF. (Auth.)
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Portfolio – et lærings- og evalueringsredskab i Diplomuddannelsen i erhvervspædagogik
DEFF Research Database (Denmark)
Højlund, Christina; Mark, Lene; Egendal, Jeppe Michael
2017-01-01
Denne artikel vil tage udgangspunkt i portfolio som læring- og evalueringsredskab i Diplomuddannelsen i Erhvervspædagogik, på valgmodulet ”Digitale teknologier i de erhvervsrettede uddannelser”. Artiklen vil undersøge, hvordan portfolioen kan understøtte de studerendes læring og refleksion, samt...... argumentere for, hvordan portfolioen kan fungere som evalueringsredskab – både formativt og summativt. Med udgangspunkt i konkrete erfaringer fra Diplomuddannelsen i Erhvervspædagogik, hvor portfolio er en del af eksamensformen, vil artiklen præsentere, analysere og diskutere hvilke anvendelsesmuligheder...... stilladsering af de lærendes arbejde med portfolio....
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Guha, Nishan; Dashwood, Alexander; Thomas, Nicholas J; Skingle, Alexander J; Sönksen, Peter H; Holt, Richard I G
2009-09-01
It is widely believed that growth hormone (GH) is abused by athletes for its anabolic and lipolytic effects. Many of the physiological effects of GH are mediated by the production of insulin-like growth factor-I (IGF-I). Both GH and IGF-I appear on the World Anti-Doping Agency list of prohibited substances. Little is known, however, about the prevalence of abuse with exogenous IGF-I. IGF-I has effects on carbohydrate, lipid and protein metabolism and some of these actions could prove beneficial to competitive athletes. No studies have demonstrated a positive effect of IGF-I on physical performance in healthy individuals but this has not yet been studied in appropriately designed trials. Two pharmaceutical preparations of IGF-I have recently become available for the treatment of growth disorders in children. This availability is likely to increase the prevalence of IGF-I abuse. Combining IGF-I with its binding protein IGFBP-3 in one preparation has the potential to reduce the side-effect profile but the adverse effects of long term IGF-I abuse are currently unknown. Detection of abuse with IGF-I is a major challenge for anti-doping authorities. It is extremely difficult to distinguish the exogenous recombinant form of the hormone from endogenously-produced IGF-I. One approach currently being investigated is based on measuring markers of GH and IGF-I action. This has already proved successful in the fight against GH abuse and, it is hoped, will subsequently lead to a similar test for detection of IGF-I abuse.
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Maj, Hubert
2015-04-01
Bilingual students in high school with bilingual units in Boguchwała have received iPads for learning English and a few subjects using CLIL (biology, basics of entrepreneurship, geography, IT and mathematics). Lessons with iPads are interesting for students for several reasons. First of all, teenagers like new technologies and using iPads for teaching helps students to learn by fun. Secondly, iPads give new possibilities of looking for knowledge about each theme. Moreover, teaching with iPads develops students' engagement. They have a chance to choose a few among over 65 000 applications for gathering and then presenting information about the lesson topic. They can easily prepare presentations, movies, cartoons, mind maps or whatever they like. Teaching students, thanks to the iPads, makes it their initiative, and the teacher can inspire them to look for the knowledge rather than disciplining pupils. But teaching with iPads is connected with many problems. For instance, there are not any examples on how to teach using these tools. It is very up-to-date technology and teachers firstly must learn the possibilities of iPads and look for new applications. It takes much time, especially at the beginning, and is difficult especially for inexperienced teachers. In addition, it is almost impossible to maintain control of the iPads for all of the students during the lesson. They can use their iPads for something unconnected with the topic of the lesson. Thirdly is lack of time - active methods (with iPads as well) are more time-consuming and it could be that they do not finish the whole program. And of course the last, but not at least, is the problem of money. Some of the applications must be paid for, and it is usually obligatory to possess a credit card. Fortunately, it is not expensive - applications usually cost a few euros and many of them are free and really good.
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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...
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Comparative analysis of secondary metabolites contents in[i] Fragaria vesca[/i] L. fruits
Directory of Open Access Journals (Sweden)
Agnieszka Najda
2014-06-01
Full Text Available [i]Fragaria vesca[/i] (wild strawberry belongs to the Rosaceae family. Besides the leaves ([i]Fragariae folium[/i] and roots ([i]Fragariae radix[/i], the aromatic fruits ([i]Fragariae fructus[/i] of wild strawberry are also herbal materials used in medicine. The aim of this study was to compare the value of phytochemical and antioxidant activity of wild strawberry fruits ([i]Fragaria vesca L[/i].. The fruits were analyzed regarding their secondary metabolites contents (flavonoids, sum of phenolic acids, tannins, anthocyanins, DPPH, depending on the origin of the raw material (from natural habitats vs. cultivation. According to the obtained results, raw material originating from natural habitats contained significantly more flavonoids (0.559 mg∙g [sup]-1[/sup] , compared to fruits harvested from cultivation (0.472 mg∙g [sup]-1[/sup] , on average. Mean concentration of phenolic acids ranged from 1.648 mg∙g[sup] -1[/sup] – 2.348 mg∙g [sup]-1[/sup] , although the wild form was characterized by higher levels of examined substances. Tannins are an important fraction of phenolic compounds; their content in studied fruits ranged from 2.2% (from cultivation – 3.0% (from natural habitats. When comparing the average contents of anthocyanins in the studied materials, it was revealed that remarkably more of these compounds were recorded in wild strawberry fruits harvested from natural habitats vs. those from cultivations: 132 mg∙100 g [sup]-1[/sup] vs. 90 mg∙100 g [sup]-1[/sup] . A difference was indicated with respect to the ability of DPPH radical reduction to diphenylpicrylhydrazine by extracts made of examined fruits.
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iSAM: An iPhone Stealth Airborne Malware
Damopoulos , Dimitrios; Kambourakis , Georgios; Gritzalis , Stefanos
2011-01-01
Part 2: Malware, Information Flow and DoS Attacks; International audience; Modern and powerful mobile devices comprise an attractive target for any potential intruder or malicious code. The usual goal of an attack is to acquire users’ sensitive data or compromise the device so as to use it as a stepping stone (or bot) to unleash a number of attacks to other targets. In this paper, we focus on the popular iPhone device.We create a new stealth and airborne malware namely iSAM able to wirelessly...
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Cardiac troponin I (CTnI level among children with epileptic seizures
Directory of Open Access Journals (Sweden)
Ahmed Anwer Attia Khattab
2014-09-01
Conclusion: Cardiac troponin I is a perfect tool for early detection of cases with myocardial dysfunction in epileptic patients – cardiac troponin I is significantly increased in children with epilepsy especially the complicated epilepsy. Cardiac injury in epileptic children is more common in patients with early onset epilepsy, positive prenatal problem, idiopathic epilepsy, abnormal imaging and EEG – elevated TnI levels may be of value in assessing the severity and eventual outcome and mortality risk of the disease in children with epilepsy.
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DEFF Research Database (Denmark)
René, Michael
Håndbog i praktisk hjælp er en opslagsbog med masser af nyttig viden om rengøring, hygiejne og opbevaring af fødevarer. Bogen indeholder afsnit om forskellige rengøringsmidler, om rengøring af forskellige materialer og om fjernelse af pletter, og der er en beskrivelse af forholdsregler i forbinde......://michaelrene.com/forfatter/ Anmeldelse: http://www.michaelrene.com/Munksgaard/AnmeldelseHaandbogIpraktiskhjaelp.png...
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Vildtudbyttet i Danmark i jagtsæsonen 2000 / 2001
DEFF Research Database (Denmark)
Asferg, T.
-udbytte-statistik end normalt. Usikkerheden skyldes især at en del jægere er registreret for usandsynligt store udbytter af især kronvildt, dåvildt og sika. Direkte henvendelse til en del af disse jægere bekræftede at der i næsten alle tilfælde var tale om fejlregistrering. Fejlene er opstå-et i forbin-del-se med den...
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Chambers, Mark L
2014-01-01
Do it all with your iMac and this bestselling For Dummies guide! You're still a little giddy from finally scoring your new iMac, and you can't wait to get started. Even if you're already in love with your iMac, it helps to have a little guidance to really get the most out of this ultimate all-in-one computer. This updated edition of iMac For Dummies is the ideal way to learn the iMac fundamentals from setting up and personalizing your machine to importing files, making FaceTime video calls, surfing the web, using your favorite programs and apps, and everything in between. Trusted Mac guru Mark
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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...
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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...
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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...
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Directory of Open Access Journals (Sweden)
Ramanzin, M.
2002-12-01
Full Text Available Studies were undertaken on the spatial overlap and habitat selection of the introduced Mouflon and the autochthon Chamois population in the Dolomiti Bellunesi National Park (Eastern Italian Alps. Mouflon distribution within the Park showed a marked seasonal shift from winter (December-May range (400-800 m a. s. I. to the summer (June-November range (1600-1800 m a. s. /J. Consequently, significant overlap with Chamois occurred only in summer. Therefore, spatial overlap and habitat selection were studied in more detail in the summer range of Mouflon. The results showed that the areas most intensively used by the two species were distinct. Both species avoided areas with rock outcrops and dense forests, Mouflon used <i>Pinus mugoi> stands and scree much more than Chamois, and conversely Chamois used more alpine pastures and <i>Larix deciduai> stands. The results therefore indicate that Chamois and Mouflon segregate spatially and use different resources.
[fr]
Nous avons étudié la superposition spatiale et la sélection de l'habitat des populations de mouflons introduits et de chamois autochtones du Parc National des Dolomites Bellunesi (Alpes italiennes orientales. La distribution du mouflon dans le parc change clairement d'une saison à l'autre (en hiver : décembremai, entre 400-800 m d'altitude ; en été : juin-novembre, entre 1600-1800 m d'altitude. Par conséquent, la population se superpose avec celle du chamois seulement en été. Nous avons alors étudié la distribution estivale du mouflon à une échelle plus fine. Nous avons pu montrer que les territoires les plus utilisés par chacune des deux espèces étaient séparés. Les deux espèces évitaient les terrains avec des escarpements ou blocs rocheux et les forêts denses, toutefois le mouflon utilisait les bosquets de <i>Pinus mugoi> et les éboulis beaucoup plus fréquemment que le chamois; ce dernier au contraire cherchait plus les pâturages alpins et les for -
Early byzantine lamellar armour from <i>Carthago Spartariai> (Cartagena, Spain
Directory of Open Access Journals (Sweden)
Vizcaíno Sánchez, Jaime
2008-12-01
Full Text Available This article presents an Early Byzantine lamellar armour, retrieved in the excavations at the quarter built over the Roman Theatre of Cartagena. The armour has close parallels with contemporary known material from the central and eastern Mediterranean or other sites, and it is an important find which increases the body of archeological evidence about Byzantine presence in <i>Spania>.
Este artículo presenta una coraza laminar protobizantina hallada en las excavaciones del barrio construido sobre el teatro romano de Cartagena. La coraza tiene estrechos paralelos con materiales contemporáneos del Mediterráneo Central y Oriental u otros lugares, y es un importante hallazgo que incrementa la nómina de evidencias arqueológicas acerca de la presencia bizantina en <i>Spania>. -
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...
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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
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Fizičko-hemijske i detonacione karakteristike nitraminskih eksploziva - RDX, HMX i CL-20
Anđelković-Lukić Mirjana
2002-01-01
U radu su prikazane fizičko-hemijske i detonacione karakteristike nitraminskih brizantnih eksploziva, heksogena i oktogena, uporedene sa osobinama novog cikličnog nitraminskog eksploziva CL-20. Novi visokobrizantni eksploziv CL-20 postoji u četiri kristalne forme, stabilne na različitim temepraturama. Ima bolje detonacione karakteristike od heksogena i oktogena veću gustinu i brzinu detonacije, all mnogo veću osetljivost na udar i trenje, reda PETN. Zbog toga se ovaj eksploziv flegmatizuje sa...
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Laron, Z; Klinger, B; Silbergeld, A
1999-01-01
Serum IGF-I levels were measured in 14 patients (9 children and 5 adults) with Laron syndrome (LS) during long-term treatment by IGF-I. Recombinant IGF-I (FK-780, Fujisawa Pharmaceutical Co. Ltd., Japan) was administered once daily subcutaneously before breakfast for 3-5 years to the children and for 9 months to the adults. The initial daily dose was 150 micrograms/kg for children and 120 micrograms/kg for adults. Before initiation of treatment the mean overnight fasting levels of serum IGF-I in the children was 3.2 +/- 0.8 nmol/l (mean +/- SEM), rising to 10 +/- 1.7 nmol/l during long-term treatment even on a dose of 120 micrograms/kg/day. The serum IGF-I levels 4 hours after injection rose from 31.2 +/- 3.5 to 48 +/- 2 nmol/l. In the adult patients, the initial basal IGF-I was 4.1 +/- 0.7 nmol/l, rising to 16.1 +/- 3.84 nmol/l after 8-9 months treatment. Serum IGF-I levels at 4 hours after injection rose in the adult patients from 24.1 +/- 5.8 up to 66.8 +/- 15.4 nmol/l. A progressively increasing half-life during long term exogenous administration of IGF-I to patients with Laron syndrome was demonstrated by following serum IGF-I dynamics after injection. Based on the fact that no antibodies to IGF-I were detected and on findings in previous studies, it is speculated that the increasing serum IGF-I levels during long-term IGF-I treatment are caused by an increase in serum IGFBP-3 induced by chronic IGF-I administration. It is concluded that treatment with IGF-I necessitates regular monitoring of serum IGF-I levels; in patients in whom the age adjusted maximal levels are exceeded, a reduction of the daily IGF-I dose is indicated to avoid undesirable effects.
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DEFF Research Database (Denmark)
Michelsen la Cour, Annette
2013-01-01
Abstract Seniorer i bevægelse Projekt Seniorer i Bevægelse var et fireårigt projekt i Københavns Kommune målrettet ældre +60, der bevægede sig mindre end anbefalet af Sundhedsstyrelsen. I perioden 2010-2014 deltog 583 borgere i fysisk træning organiseret omkring sociale netværker, heraf ca. 25...
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DEFF Research Database (Denmark)
Michelsen la Cour, Annette
2014-01-01
Projekt Seniorer i Bevægelse var et fireårigt projekt i Københavns Kommune målrettet ældre +60, der bevægede sig mindre end anbefalet af Sundhedsstyrelsen. I perioden 2010-2014 deltog 583 borgere i fysisk træning organiseret omkring sociale netværker, ca. 25% blev rekrutteret i et samarbejde med en...
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Modeling Li I and K I sensitivity to Pleiades activity
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
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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
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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....
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Biersdorfer, J D
2010-01-01
With iPod and iTunes, Apple's gotten the world hooked on portable music, pictures, and videos. One thing they haven't delivered, though, is an easy guide for getting the most from your sleek little entertainment center. Enter iPod: The Missing Manual, 5th Edition-a book as breathtaking and satisfying as its subject. Our latest edition thoroughly covers the redesigned iPod Nanos, the video iPod, the tiny Shuffle and the overhauled iTunes 7. Each custom-designed page sports easy-to-follow color graphics, crystal-clear explanations, and guidance on the most useful things your iPod can do. Topic
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Energy Technology Data Exchange (ETDEWEB)
Frechou, C
2000-07-01
{sup 129}I, is a natural long-lived isotope, with a half-life of 15,7 million years, also artificially produced in nuclear power plant. It is then released in the liquid and gaseous effluents of the nuclear fuel reprocessing plants. {sup 129}I is integrated in all biological compartments at different activity levels, depending on their distance from the emission source and their ability to metabolize iodine. Performances of the different {sup 129}I and {sup 129}I/{sup 127}I measurement techniques available: Radiochemical Neutron Activation Analysis, Accelerator Mass Spectrometry, direct {gamma}-X spectrometry and liquid scintillation were evaluated. Associated radiochemical preparation steps of the two first techniques were optimized and adapted to the characteristics of the major environmental matrices. In a first step, the radiochemical protocols were developed and validated. In a second step, intercomparison exercises have been lead on various environmental samples presenting different {sup 129}I activity levels. They showed the good agreement between the results given by the three techniques on different environmental matrices with activities between 0,2 and 200 Bq.kg{sup -1} dry weight. As a conclusion, a methodology for the measurement of {sup 129}I and {sup 129}I/{sup 127}I ratio in environmental samples is proposed. It includes a decisional diagram taking into account the characteristics of the matrices, the detection limits and the answer delay. A study on the losses of {sup 129}I during the calcination of an algae was lead by direct {gamma}-X spectrometry and application studies were made to measure {sup 129}I levels in different biological compartments issued from various locations: {sup 129}I activity interspecific variation in different species of seaweeds from the French channel coast under the relative influence of La Hague, {sup 129}I levels in bovine thyroids from the Cotentin area and {sup 129}I in vegetal samples collected around the nuclear
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International Nuclear Information System (INIS)
Flier, J.S.; Usher, P.; Moses, A.C.
1986-01-01
Insulin and insulin-like growth factor type I (IGF-I) stimulate an overlapping spectrum of biological responses in human skin fibroblasts. Although insulin and IGF-I are known to stimulate the incorporation of [ 3 H]thymidine into DNA in these cells, the identify of the receptor(s) that mediates this effect has not been fully clarified. The mouse anti-human IGF-I receptor antibody αIR-3 binds with specificity to IGF-I but not to insulin receptors in human placental membranes; it also specifically inhibits the binding of 125 I-labeled IGF-I but not 125 I-labeled insulin to suspensions of human skin fibroblasts in a dose-dependent manner. αIR-3 competitively inhibits IGF-I-mediated stimulation of [ 3 H]thymidine incorporation into DNA. This inhibition is dependent on the concentration of αIR-3 and in the presence of a fixed antibody concentration can be partially overcome by high concentrations of IGF-I. In contrast, at concentrations of 3 H]thymidine incorporation is not inhibited by αIR-3. However, the incremental effects of higher concentrations (> 1 μg/ml) of insulin on [ 3 H]thymidine incorporation are inhibited by αIR-3. αIR-3 is a highly specific antagonist of IGF-I receptor-mediated mitogenesis in human skin fibroblasts. By using this antibody, it is shown directly that insulin can act through the IGF-I receptor to stimulate DNA synthesis but can also activate this effect through the insulin receptor itself
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The autoinhibitory CARD2-Hel2i Interface of RIG-I governs RNA selection.
Ramanathan, Anand; Devarkar, Swapnil C; Jiang, Fuguo; Miller, Matthew T; Khan, Abdul G; Marcotrigiano, Joseph; Patel, Smita S
2016-01-29
RIG-I (Retinoic Acid Inducible Gene-I) is a cytosolic innate immune receptor that detects atypical features in viral RNAs as foreign to initiate a Type I interferon signaling response. RIG-I is present in an autoinhibited state in the cytoplasm and activated by blunt-ended double-stranded (ds)RNAs carrying a 5' triphosphate (ppp) moiety. These features found in many pathogenic RNAs are absent in cellular RNAs due to post-transcriptional modifications of RNA ends. Although RIG-I is structurally well characterized, the mechanistic basis for RIG-I's remarkable ability to discriminate between cellular and pathogenic RNAs is not completely understood. We show that RIG-I's selectivity for blunt-ended 5'-ppp dsRNAs is ≈3000 times higher than non-blunt ended dsRNAs commonly found in cellular RNAs. Discrimination occurs at multiple stages and signaling RNAs have high affinity and ATPase turnover rate and thus a high katpase/Kd. We show that RIG-I uses its autoinhibitory CARD2-Hel2i (second CARD-helicase insertion domain) interface as a barrier to select against non-blunt ended dsRNAs. Accordingly, deletion of CARDs or point mutations in the CARD2-Hel2i interface decreases the selectivity from ≈3000 to 150 and 750, respectively. We propose that the CARD2-Hel2i interface is a 'gate' that prevents cellular RNAs from generating productive complexes that can signal. © The Author(s) 2015. Published by Oxford University Press on behalf of Nucleic Acids Research.
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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...
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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...
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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...
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Daniel, Steven F
2012-01-01
Each chapter will take you through a new major feature of iOS 5. You will learn how to integrate each feature into your applications. If you ever wanted to learn about the latest features of iOS 5 and learn how to incorporate Twitter, iCloud and Core Image framework effects functionality into your applications, then this book is for you. You should have a good knowledge of programming experience with Objective-C, and have used Xcode 4. iPhone programming experience is not required.
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Energy Technology Data Exchange (ETDEWEB)
Gould, Emily N. [Univ. of Tennessee College of Veterinary Medicine, Knoxville, TN (United States); Giannone, Richard [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Kania, Stephen A. [The Univ. of Tennessee College of Veterinary Medicine, Knoxville, TN (United States); Tolbert, M. Katherine [Univ. of Tennessee College of Veterinary Medicine, Knoxville, TN (United States)
2017-08-01
<i>Tritrichomonas foetusi> (<i>T. foetusi>) is a flagellated protozoan parasite that is recognized as a significant cause of diarrhea in domestic cats with a prevalence rate as high as 30%. No drugs have been shown to consistently eliminate <i>T. foetusi> infection in all cats. Cysteine proteases (CPs) have been identified as mediators of <i>T. foetusi>-induced adhesion-dependent cytotoxicity to the intestinal epithelium. These CPs represent novel targets for the treatment of feline trichomonosis. However, cats also produce CPs that are part of life-critical systems. Thus, parasitic CPs need to be selectively targeted to reduce the potential for host toxicity. Previous studies have demonstrated the importance of a specific CP, CP30, in mediating bovine and human trichomonad cytopathogenicity. This CP has also recently been identified in feline <i>T. foetusi>, although the function of this protease in the feline genotype remains unknown. Furthermore, the study objectives were to characterize the presence of CP30 in feline <i>T. foetusi> isolates and to evaluate the effect of targeted inhibition of CP30 on feline <i>T. foetusi>-induced adhesion dependent cytotoxicity.
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<i>Liber Nycholayi>. La leyenda de Mahoma y el cardenal Nicolás
Directory of Open Access Journals (Sweden)
González Muñoz, Fernando
2004-06-01
Full Text Available The aim of this paper is to offer a critical edition, with translation and commentary, of <i>Liber Nycholayi>, an anonymously authored Latin text of Italian origin, probably Roman, which could be dated back to the second half of the thirteenth century. <i>Liber Nycholayi> presents a curious version of the legend of Mahoma, according to which he would have originally been a Roman cardinal called Nicholaus, whose frustrated ambitions to papacy led him to found a new religion in revenge. The text combines data on the Islamic doctrines and practices with anachronisms and legendary details further verified in other mediaeval texts. This edition has been drawn from the two existing manuscripts of the work, the <i>Parisinus BN lat. 14503i> and the <i>Vaticanus Reginensis Latinus 627i>.
El presente trabajo ofrece una edición crítica, con traducción y estudio, del <i>Liber Nycholayi>, un texto latino anónimo de procedencia italiana, probablemente romana, que puede datarse en la segunda mitad del siglo XlIl. El <i>Liber Nycholayi> presenta una curiosa versión de la leyenda de Mahoma, según la cual éste habría sido en origen un cardenal romano llamado Nicolás que, frustrado en sus ambiciones por acceder al papado, fundó una nueva religión a modo de venganza. El texto combina datos exactos sobre las doctrinas y prácticas islámicas con anacronismos y detalles legendarios atestiguados también en otros textos medievales. La edición se ha confeccionado a partir de los dos manuscritos que transmiten la obra, el Parisinus BN lat. 14503 y el Vaticanus Reginensis Latinus 627. -
iQuantitator: A tool for protein expression inference using iTRAQ
Directory of Open Access Journals (Sweden)
Comte-Walters Susana
2009-10-01
Full Text Available Abstract Background Isobaric Tags for Relative and Absolute Quantitation (iTRAQ™ [Applied Biosystems] have seen increased application in differential protein expression analysis. To facilitate the growing need to analyze iTRAQ data, especially for cases involving multiple iTRAQ experiments, we have developed a modeling approach, statistical methods, and tools for estimating the relative changes in protein expression under various treatments and experimental conditions. Results This modeling approach provides a unified analysis of data from multiple iTRAQ experiments and links the observed quantity (reporter ion peak area to the experiment design and the calculated quantity of interest (treatment-dependent protein and peptide fold change through an additive model under log transformation. Others have demonstrated, through a case study, this modeling approach and noted the computational challenges of parameter inference in the unbalanced data set typical of multiple iTRAQ experiments. Here we present the development of an inference approach, based on hierarchical regression with batching of regression coefficients and Markov Chain Monte Carlo (MCMC methods that overcomes some of these challenges. In addition to our discussion of the underlying method, we also present our implementation of the software, simulation results, experimental results, and sample output from the resulting analysis report. Conclusion iQuantitator's process-based modeling approach overcomes limitations in current methods and allows for application in a variety of experimental designs. Additionally, hypertext-linked documents produced by the tool aid in the interpretation and exploration of results.
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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...
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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...
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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...
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Directory of Open Access Journals (Sweden)
Susanna, A.
2003-12-01
Full Text Available Generic delineation within the <i>Arctium> group (<i>Compositae, Carduceae-Carduinaei>, formed by the genera <i>Arctium, Cousinia, Hypacanthiumi> and <i>Schamalhausenia>, has proven a complicated task. In particular, the precise limits between <i>Arctium> and <i>Cousinia> are very difficult to establish. Therefore, we have carried out a molecular survey of DNA sequences of two regions, the chloroplast gene <i>mat>K and the nuclear-ribosomal spacers ITS 1 and 2, of a representation of all the genera of the group (in the case of <i>Cousinia>, centered in the species more obviously related to <i>Arctiium>. Our results show a precise correlation between molecular phylogeny and two very important characters, pollen type and chromosome numbers: all the investigated species with the <i>Arctiastrum> pollen type and x= 18, characteristics of <i>Arctium> sensu stricto, form a monophyletic clade, sister of another monophyletic clade formed by all the investigated species of <i>Cousinia> sensu slricto. However, the resulting "Arctioid" clade cannot be defined on macroscopic morphologic characters, because the main trait for segregating <i>Arctium> and <i>Cousinia>, the spiny pinnatifid-pinnatisect leaves of <i>Cousinia>, is adaptative and of scarce systematic relevance. In fact, our results suggest that spines have appeared at least in two different lineages: the genera <i>Hypacanthium> and <i>Schamalhausenia>, spiny and thus morphologically closer to <i>Cousinia>, are unambiguously related to the unarmed genus <i>Arctium>. An hypothesis on the evolution of morphology, pollen and chromosome numbers in the group is formulated. The systematic implications of this incongruence between molecular, pollen and karyology, on the one hand, and morphology, on the other hand, are evaluated. Some possible solutions are proposed, but none of them is totally satisfactory: more studies are necessary with the inclusion
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Collective properties of the odd-mass I nuclei: 123,125,127I
Shroy, R. E.; Gordon, D. M.; Gai, M.; Fossan, D. B.; Gaigalas, A. K.
1982-09-01
The high-spin states of 123,125,127I have been investigated via the ASn(6Li, 3n)A+3I reactions to study the collective properties of the odd-mass I isotopes. In-beam measurements of γ-ray excitations, γ-γ coincidences, γ-ray angular distributions, and pulsed beam-γ timing were performed with Ge detectors to determine level energies, decay schemes, γ-ray multipolarities, Jπ assignments, and lifetime information. A similar study of the 117,119,121I isotopes is reported in the following paper. Two collective features have been identified in these odd-mass I nuclei. Systematic ΔJ=1 bands built on low-lying 92+ proton-hole (4p-1h) states were observed. The 92+ bandheads, that involve the excitation of a 1g92 proton across the Z=50 shell, drop to very low energies near the middle of the neutron shell. The properties of the 92+ proton-hole states for all of the odd-mass I isotopes are presented and related to the systematic information for the proton-hole states in the entire Z>50 transition region. Systematic ΔJ=2 bands built on 112- (1h112 quasiproton) states, on 72+ (1g72 quasiproton) states, and on 52+ (2d52 quasiproton) states were also observed. The ΔJ=2 band spacings generally follow the spacings of the Te-core ground-state bands with the exception of the 112- ΔJ=2 bands, for which the spacings decrease significantly relative to those for the Te cores as A decreases. These systematic properties are discussed in terms of several theoretical approaches to the onset of collectivity in transitional nuclei. An isomer at 2660 keV in 123I was observed to have a mean lifetime τ=38+/-3 ns. NUCLEAR REACTIONS 120-124Sn(6Li, 3n)123-127I measured γ-γ coincidences, γ(E, θ, t) deduced level schemes in odd-mass 123-127I, γ multipolarities, Jπ, T12. Enriched targets, Ge(Li) detectors.
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Synthesis of the 123I- and 125I-labeled cholinergic nerve marker (-)-5-iodobenzovesamicol
International Nuclear Information System (INIS)
Van Dort, M.E.; Jung, Y.-W.; Gildersleeve, D.L.; Hagen, C.A.; Kuhl, D.E.; Wieland, D.M.
1993-01-01
The highly toxic curaremimetic and cholinergic neuron marker (-)-5-iodobenzovesamicol (IBVM) has been labeled with iodine-125 and iodine-123. [ 125 I]IBVM, suitable for animal distribution and ex vivo autoradiographic studies, was synthesized by solid-state exchanger; isolated yields were 65-89% with specific activities in the range of 130-200 Ci/mmol. The synthesis of no-carrier-added (-)-5-[ 125 I]IBVM from the corresponding chiral (-)-5-(tri-n-butyltin) derivative using Na 125 I was evaluated using the oxidants H 2 O 2 , peracetic acid and chloramine-T. Both peracetic acid and chloramine-T gave good yields (70-95%). However, when Na 123 I was utilized, acceptable yields of [ 123 I]IBVM were obtained only with chloramine-T. Distribution analyses of [ 125 I]IBVM and [ 123 I]IBVM in mice 4 h following intravenous administration show essentially equivalent concentrations of the two tracers in the four brain regions sampled. The exceptionally high specific activity of [ 123 I]IBVM has made possible the evaluation of this radiotracer in humans. (Author)
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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...
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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...