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Sample records for dysautonomia expanding spectrum

  1. Dysautonomia in Autism Spectrum Disorder: Case Reports of a Family with Review of the Literature

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    Derrick Lonsdale

    2011-01-01

    Full Text Available Case histories of a mother and her two children are reported. The mother was a recovered alcoholic. She and her two children, both of whom had symptoms that are typical of autistic spectrum disorder, had dysautonomia. All had intermittently abnormal erythrocyte transketolase studies indicating abnormal thiamine pyrophosphate homeostasis. Both children had unusual concentrations of urinary arsenic. All had symptomatic improvement with diet restriction and supplementary vitamin therapy but quickly relapsed after ingestion of sugar, milk, or wheat. The stress of a heavy metal burden, superimposed on existing genetic or epigenetic risk factors, may be important in the etiology of autism spectrum disorder when in combination. Dysautonomia has been associated with several diseases, including autism, without a common etiology. It is hypothesized that oxidative stress results in loss of cellular energy and causes retardation of hard wiring of the brain in infancy, affecting limbic system control of the autonomic nervous system.

  2. Cat Scratch Disease: Expanded Spectrum

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    Aziz, Hassan A.; Plesec, Thomas P.; Sabella, Camille; Udayasankar, Unni K.; Singh, Arun D.

    2016-01-01

    Background To expand the spectrum of ophthalmic manifestations in cat scratch disease. Methods Case report. Results A 7-year-old male was referred for evaluation of his left optic disc after failing vision screening test at school. His visual acuity was 20/20 OD and light perception OS. Fundus examination showed a left optic disc lesion associated with an exudative retinal detachment and vitreous seeding. Ultrasonography revealed a 7 × 7.5 × 3.8 mm lesion with a possible 6.3 mm of retrolaminar extension into the substance of the optic nerve. Brain MRI did not show evidence of optic nerve involvement but revealed a 6-mm nodule of the pineal gland suggestive of a pineoblastoma. Enucleation was performed and histopathology revealed a suppurative granulomatous inflammation suggestive of Bartonella infection. Upon further questioning, the patient had recent exposure to kittens with areas of cat scratches along both of his arms. He was subsequently referred to and treated with a 2-week course of trimethoprim-sulfamethoxazole and rifampin by the pediatric infectious disease specialist. Repeat brain MRI showed interval total resolution of enlarged pineal gland. Conclusion: Optic nerve granulomas are a rare presentation of cat scratch disease and could potentially masquerade as retinoblastoma. PMID:27843905

  3. Trichorhinophalangeal syndrome II, expanding the clinical spectrum

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    Rabah M. Shawky

    2015-01-01

    Full Text Available We report a 4.5 year old Egyptian male child, fourth in the order of birth of healthy remote consanguineous parents. He has typical facial as well as skeletal features of Trichorhinophalangeal syndrome (TRPS II. The facial features included bilateral downward slanting palpebral fissures, bulbous nose, long filtrum, retromicrognathia, sparse hair in the scalp and thick eyebrows. The skeletal features included retarded bone age, cone shaped epiphyses of the phalanges and multiple exostoses. The patient has also growth retardation, moderate mental retardation and hyperlaxity of the right knee joint. However our patient has some features not reported in TRPS II patients. These included bilateral partial ptosis, long eye lashes, preauricular skin tag, short 2nd right finger, short metacarpals of both thumbs. So we have to expand the clinical spectrum. Karyotype demonstrated 46,XY,del 8(q23.3-q24.1.

  4. Optic neuropathy in familial dysautonomia.

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    Groom, M; Kay, M D; Corrent, G F

    1997-06-01

    Optic atrophy, which is indicative of a CNS disorder, is a rarely described manifestation of familial dysautonomia (Riley-Day syndrome). As these patients are now living longer, the prevalence of optic neuropathy also may be increasing. We present a man with familial dysautonomia and visual loss resulting from optic atrophy and visual field defect suggestive of chiasmal pathology.

  5. Expanding the solar spectrum used by photosynthesis

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Min; Blankenship, R. E.

    A limiting factor for photosynthetic organisms is their light-harvesting efficiency, that is the efficiency of their conversion of light energy to chemical energy. Small modifications or variations of chlorophylls allow photosynthetic organisms to harvest sunlight at different wavelengths. Oxygenic photosynthetic organisms usually utilize only the visible portion of the solar spectrum. The cyanobacterium Acaryochloris marina carries out oxygenic photosynthesis but contains mostly chlorophyll d and only traces of chlorophyll a. Chlorophyll d provides a potential selective advantage because it enables Acaryochloris to use infrared light (700–750 nm) that is not absorbed by chlorophyll a. Recently, an even more red-shifted chlorophyll termed chlorophyll f has been reported. Here, we discuss using modified chlorophylls to extend the spectral region of light that drives photosynthetic organisms.

  6. Genetics Home Reference: familial dysautonomia

    Science.gov (United States)

    ... during infancy. Early signs and symptoms include poor muscle tone (hypotonia), feeding difficulties, poor growth, lack of tears, frequent lung infections, and difficulty maintaining body temperature. Older infants and young children with familial dysautonomia may hold their breath ...

  7. Expanding the mutation and clinical spectrum of Roberts syndrome.

    Science.gov (United States)

    Afifi, Hanan H; Abdel-Salam, Ghada M H; Eid, Maha M; Tosson, Angie M S; Shousha, Wafaa Gh; Abdel Azeem, Amira A; Farag, Mona K; Mehrez, Mennat I; Gaber, Khaled R

    2016-07-01

    Roberts syndrome and SC phocomelia syndrome are rare autosomal recessive genetic disorders representing the extremes of the spectrum of severity of the same condition, caused by mutations in ESCO2 gene. We report three new patients with Roberts syndrome from three unrelated consanguineous Egyptian families. All patients presented with growth retardation, mesomelic shortening of the limbs more in the upper than in the lower limbs and microcephaly. Patients were subjected to clinical, cytogenetic and radiologic examinations. Cytogenetic analysis showed the characteristic premature separation of centromeres and puffing of heterochromatic regions. Further, sequencing of the ESCO2 gene identified a novel mutation c.244_245dupCT (p.T83Pfs*20) in one family besides two previously reported mutations c.760_761insA (p.T254Nfs*27) and c.764_765delTT (p.F255Cfs*25). All mutations were in homozygous state, in exon 3. The severity of the mesomelic shortening of the limbs and craniofacial anomalies showed variability among patients. Interestingly, patient 1 had abnormal skin hypopigmentation. Serial fetal ultrasound examinations and measurements of long bones diagnosed two affected fetuses in two of the studied families. A literature review and case comparison was performed. In conclusion, we report a novel ESCO2 mutation and expand the clinical spectrum of Roberts syndrome.

  8. Current Treatments in Familial Dysautonomia

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    Palma, Jose-Alberto; Kaufmann, Lucy; Fuente, Cristina; Percival, Leila; Mendoza, Carlos; Kaufmann, Horacio

    2014-01-01

    Introduction Familial dysautonomia (FD) is a rare hereditary sensory and autonomic neuropathy (type III). The disease is caused by a point mutation in the IKBKAP gene that affects the splicing of the elongator-1 protein (also known as IKAP). Patients have dramatic blood pressure instability due to baroreflex failure, chronic kidney disease, and impaired swallowing leading to recurrent aspiration pneumonia, which results in chronic lung disease. Diminished pain and temperature perception results in neuropathic joints and thermal injuries. Impaired proprioception leads to gait ataxia. Optic neuropathy and corneal opacities lead to progressive visual loss. Areas covered This article reviews current therapeutic strategies for the symptomatic treatment of FD, as well as the potential of new gene modifying agents. Expert opinion Therapeutic focus on FD is centered on reducing the catecholamine surges caused by baroreflex failure. Managing neurogenic dysphagia with effective protection of the airway passages and prompt treatment of aspiration pneumonias is necessary to prevent respiratory failure. Sedative medications should be used cautiously due to risk of respiratory depression. Non-invasive ventilation during sleep effectively manages apneas and prevents hypercapnia. Clinical trials of compounds that increase levels of IKAP (ELP-1) are underway and will determine whether they can reverse or slow disease progression. PMID:25323828

  9. Familial Dysautonomia: Mechanisms and Models

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    Paula Dietrich

    Full Text Available Abstract Hereditary Sensory and Autonomic Neuropathies (HSANs compose a heterogeneous group of genetic disorders characterized by sensory and autonomic dysfunctions. Familial Dysautonomia (FD, also known as HSAN III, is an autosomal recessive disorder that affects 1/3,600 live births in the Ashkenazi Jewish population. The major features of the disease are already present at birth and are attributed to abnormal development and progressive degeneration of the sensory and autonomic nervous systems. Despite clinical interventions, the disease is inevitably fatal. FD is caused by a point mutation in intron 20 of the IKBKAP gene that results in severe reduction in expression of IKAP, its encoded protein. In vitro and in vivo studies have shown that IKAP is involved in multiple intracellular processes, and suggest that failed target innervation and/or impaired neurotrophic retrograde transport are the primary causes of neuronal cell death in FD. However, FD is far more complex, and appears to affect several other organs and systems in addition to the peripheral nervous system. With the recent generation of mouse models that recapitulate the molecular and pathological features of the disease, it is now possible to further investigate the mechanisms underlying different aspects of the disorder, and to test novel therapeutic strategies.

  10. Familial dysautonomia (Riley-Day syndrome).

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    Tonholo-Silva, E R; Takahashi, S I; Yoshinaga, L

    1994-03-01

    Familial dysautonomia, also known as Riley-Day syndrome, is a disorder of autonomic nervous system with an autosomal recessive mode of inheritance. Reduction and/or loss of unmyelinated and small myelinated fibers is found, as reduction of dopamine beta-hydroxylase in blood. The diagnosis is based on clinical features: diminished lacrimation, insensitivity to pain, poor temperature control, abolished deep tendon reflexes, postural hypotension, vomiting attacks, poor motor coordination, and mental retardation. The treatment is symptomatic and many children die during the first years of life, usually as a result of repeated aspiration pneumonia. We report the case of a 1 year-old child with familial dysautonomia.

  11. Mother-induced hypertension in familial dysautonomia.

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    Norcliffe-Kaufmann, Lucy; Palma, Jose-Alberto; Kaufmann, Horacio

    2016-02-01

    Here we report the case of a patient with familial dysautonomia (a genetic form of afferent baroreflex failure), who had severe hypertension (230/149 mmHg) induced by the stress of his mother taking his blood pressure. His hypertension subsided when he learnt to measure his blood pressure without his mother's involvement. The case highlights how the reaction to maternal stress becomes amplified when catecholamine release is no longer under baroreflex control.

  12. Probing the Scale Invariance of the Inflationary Power Spectrum in Expanding Dipolar Condensates

    CERN Document Server

    Chä, Seok-Yeong

    2016-01-01

    We consider an analogue de Sitter cosmos in an expanding quasi-two-dimensional Bose-Einstein condensate, with dominant dipole-dipole interactions between the atoms or molecules in the ultracold gas. It is demonstrated that a hallmark signature of inflationary cosmology, the scale invariance of the power spectrum of inflaton field correlations, experiences strong modifications when at the initial stage of expansion the excitation spectrum displays a roton minimum. Dipolar quantum gases thus furnish a viable laboratory tool to experimentally investigate, with well-defined and controllable initial conditions, whether primordial oscillation spectra deviating from Lorentz invariance at trans-Planckian momenta violate standard predictions of inflationary cosmology.

  13. Voltage-gated potassium channelopathy: an expanding spectrum of clinical phenotypes.

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    Shribman, Sam; Patani, Rickie; Deeb, Jacquie; Chaudhuri, Abhijit

    2013-01-10

    Autoimmune voltage-gated potassium channelopathies represent a wide and expanding spectrum of neurological conditions. We present a case demonstrating the phenotypic heterogeneity of antivoltage-gated potassium channels (VGKC)-associated disorders. Such cases may easily be dismissed as functional disorders at first presentation. We propose that there must remain a high index of suspicion for antiVGKC-associated disorders in cases where there are transient neurological disturbances in atypical spatial and temporal distributions.

  14. [Riley-Day syndrome (familial dysautonomia)].

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    Kita, K

    1992-04-01

    Riley-Day syndrome (RDS, familial dysautonomia) is reviewed from a viewpoint of autonomic disturbance. RDS shows pandysautonomia, including alacrima, orthostatic hypotension, gastrointestinal paresis, and paroxysmal hyperautonomic state, such as hypertension, vomiting crisis, and blotchy erythema. Sensory disturbances, including absence of taste and pain sensation, are common. Fungiform papillae on the tongue are sparse. Tests of autonomic function reveal postganglionic dysfunction. Sural nerve biopsy reveals depletion of small myelinated fibers and unmyelinated fibers, which corresponds well with the sensory and autonomic disturbances. As to the pathogenesis of RDS, dysgenesis of neurons from the neural crest or abnormality of nerve growth factor has been suggested, but this remains undetermined.

  15. Expanding the phenotypic and mutational spectrum in microcephalic osteodysplastic primordial dwarfism type I.

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    Abdel-Salam, Ghada M H; Abdel-Hamid, Mohamed S; Issa, Mahmoud; Magdy, Ahmed; El-Kotoury, Ahmed; Amr, Khalda

    2012-06-01

    Mutations in the RNU4ATAC gene cause microcephalic osteodysplastic primordial dwarfism type I. It encodes U4atac, a small nuclear RNA that is a component of the minor spliceosome. Six distinct mutations in 30 patients diagnosed as microcephalic osteodysplastic primordial dwarfism type I have been described. We report on three additional patients from two unrelated families presenting with a milder phenotype of microcephalic osteodysplastic primordial dwarfism type I and metopic synostosis. Patient 1 had two novel heterozygous mutations in the 3' prime stem-loop, g.66G > C and g.124G > A while Patients 2 and 3 had a homozygous mutation g.55G > A in the 5' prime stem-loop. Although they manifested the known spectrum of clinical features of microcephalic osteodysplastic primordial dwarfism type I, they lacked evidence of severe developmental delay and neurological symptoms. These findings expand the mutational and phenotypic spectrum of this syndrome. Copyright © 2012 Wiley Periodicals, Inc.

  16. Analysis of inborn errors of metabolism:disease spectrum for expanded newborn screening in Hong Kong

    Institute of Scientific and Technical Information of China (English)

    Han-Chih Hencher Lee; Wai-Kwan Siu; Sammy Pak-Lam Chen; Chun-Yiu Law; Morris Hok-Leung Tai; Sidney Tam; Albert Yan-Wo Chan; Chloe Miu Mak; Ching-Wan Lam; Yuet-Ping Yuen; Angel On-Kei Chan; Chi-Chung Shek; Tak-Shing Siu; Chi-Kong Lai; Chor-Kwan Ching

    2011-01-01

    Background Data of classical inborn errors of metabolism (IEM) of amino acids,organic acids and fatty acid oxidation are largely lacking in Hong Kong,where mass spectrometry-based expanded newborn screening for IEM has not been initiated. The current study aimed to evaluate the approximate incidence,spectrum and other characteristics of classical IEM in Hong Kong,which would be important in developing an expanded newborn screening program for the local area.Methods The laboratory records of plasma amino acids,plasma acylcarnitines and urine organic acids analyses from year 2005 to 2009 inclusive in three regional chemical pathology laboratories providing biochemical and genetic diagnostic services for IEM were retrospectively reviewed.Results Among the cohort,43 patients were diagnosed of IEM,including 30 cases (69%) of amino acidemias(predominantly citrin deficiency,hyperphenylalaninemia due to 6-pyruvoyl-tetrahydropterin synthase deficiency and tyrosinemia type Ⅰ),5 cases (12%) of organic acidemias (predominantly holocarboxylase synthetase deficiency) and 8cases (19%) of fatty acid oxidation defects (predominantly carnitine-acylcarnitine translocase deficiency). The incidence of classical IEM in Hong Kong was roughly estimated to be at least 1 case per 4122 lives births,or 0.243 cases per 1000live births. This incidence is similar to those reported worldwide,including the mainland of China. The estimated incidence of hyperphenylalaninemia was 1 in 29 542 live births.Conclusions Our data indicate that it is indisputable for the introduction of expanded newborn screening program in Hong Kong. Since Hong Kong is a metropolitan city,a comprehensive expanded newborn screening program and referral system should be available to serve the neonates born in the area.

  17. MOMO Syndrome with Holoprosencephaly and Cryptorchidism: Expanding the Spectrum of the New Obesity Syndrome

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    Sheetal Sharda

    2011-01-01

    Full Text Available There are multiple genetic disorders with known or unknown etiology grouped under obesity syndromes. Inspite of having multisystem involvement and often having a characteristic presentation, the understanding of the genetic causes in the majority of these syndromes is still lacking. The common obesity syndromes are Bardet-Biedl, Prader-Willi, Alstrom, Albright's hereditary osteodystrophy, Carpenter, Rubinstein-Taybi, Fragile X, and Börjeson-Forssman-Lehman syndrome. The list is ever increasing as new syndromes are being added to it. One of the recent additions is MOMO syndrome, with about five such cases being reported in literature. Expanding the spectrum of clinical features, we report the first case of MOMO syndrome from India with lobar variant of holoprosencephaly and cryptorchidism, which have not been reported previously.

  18. MOMO Syndrome with Holoprosencephaly and Cryptorchidism: Expanding the Spectrum of the New Obesity Syndrome.

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    Sharda, Sheetal; Panigrahi, Inusha; Marwaha, Ram Kumar

    2011-01-01

    There are multiple genetic disorders with known or unknown etiology grouped under obesity syndromes. Inspite of having multisystem involvement and often having a characteristic presentation, the understanding of the genetic causes in the majority of these syndromes is still lacking. The common obesity syndromes are Bardet-Biedl, Prader-Willi, Alstrom, Albright's hereditary osteodystrophy, Carpenter, Rubinstein-Taybi, Fragile X, and Börjeson-Forssman-Lehman syndrome. The list is ever increasing as new syndromes are being added to it. One of the recent additions is MOMO syndrome, with about five such cases being reported in literature. Expanding the spectrum of clinical features, we report the first case of MOMO syndrome from India with lobar variant of holoprosencephaly and cryptorchidism, which have not been reported previously.

  19. The expanded spectrum of neuromyelitis optica: evidences for a new definition

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    Marco A Lana-Peixoto

    2012-10-01

    Full Text Available Neuromyelitis optica (NMO has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM. Identification of aquaporin-4 antibody (AQP4-IgG has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM. Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

  20. On the cosmic ray spectrum from type II Supernovae expanding in their red giant presupernova wind

    CERN Document Server

    Cardillo, Martina; Blasi, Pasquale

    2015-01-01

    While from the energetic point of view SNRs are viable sources of Galactic CRs, the issue of whether they can accelerate protons up to PeV remains unsolved. Here we discuss particle acceleration at the forward shock of SN and discuss the possibility that the escaping particle current may excite a non-resonant instability that in turn leads to the formation of resonant modes confining particles close to the shock and increasing the maximum energy. This mechanism works throughout the expansion of the SN explosion, from the ejecta dominated (ED) to the Sedov-Taylor (ST) phase. Because of their higher explosion rate,we focus on type II SNae expanding in the slow, dense red supergiant wind. When the explosion occurs in such winds, the transition between the ED and the ST phase is likely to take place within a few tens of years. As a result, the spectrum of accelerated particles shows a break in the slope, at the maximum energy (Em) achieved at the beginning of the ST phase. Above this energy, the spectrum becomes ...

  1. Gabapentin in the management of dysautonomia following severe traumatic brain injury: a case series

    DEFF Research Database (Denmark)

    Baguley, Ian J; Heriseanu, Roxana E; Gurka, Joseph A;

    2007-01-01

    The pharmacological management of dysautonomia, otherwise known as autonomic storms, following acute neurological insults, is problematic and remains poorly researched. This paper presents six subjects with dysautonomia following extremely severe traumatic brain injury where gabapentin controlled...

  2. Expanding the mutation spectrum in ICF syndrome: Evidence for a gender bias in ICF2.

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    van den Boogaard, M L; Thijssen, P E; Aytekin, C; Licciardi, F; Kıykım, A A; Spossito, L; Dalm, V A S H; Driessen, G J; Kersseboom, R; de Vries, F; van Ostaijen-Ten Dam, M M; Ikinciogullari, A; Dogu, F; Oleastro, M; Bailardo, E; Daxinger, L; Nain, E; Baris, S; van Tol, M J D; Weemaes, C; van der Maarel, S M

    2017-01-27

    Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare, genetically heterogeneous, autosomal recessive disorder. Patients suffer from recurrent infections caused by reduced levels or absence of serum immunoglobulins. Genetically, 4 subtypes of ICF syndrome have been identified to date: ICF1 (DNMT3B mutations), ICF2 (ZBTB24 mutations), ICF3 (CDCA7 mutations), and ICF4 (HELLS mutations). To study the mutation spectrum in ICF syndrome. Genetic studies were performed in peripheral blood lymphocyte DNA from suspected ICF patients and family members. We describe 7 ICF1 patients and 6 novel missense mutations in DNMT3B, affecting highly conserved residues in the catalytic domain. We also describe 5 new ICF2 patients, one of them carrying a homozygous deletion of the complete ZBTB24 locus. In a meta-analysis of all published ICF cases, we observed a gender bias in ICF2 with 79% male patients. The biallelic deletion of ZBTB24 provides strong support for the hypothesis that most ICF2 patients suffer from a ZBTB24 loss of function mechanism and confirms that complete absence of ZBTB24 is compatible with human life. This is in contrast to the observed early embryonic lethality in mice lacking functional Zbtb24. The observed gender bias seems to be restricted to ICF2 as it is not observed in the ICF1 cohort. Our study expands the mutation spectrum in ICF syndrome and supports that DNMT3B and ZBTB24 are the most common disease genes. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  3. On the cosmic ray spectrum from type II Supernovae expanding in their red giant presupernova wind

    Directory of Open Access Journals (Sweden)

    Cardillo Martina

    2015-01-01

    Full Text Available While from the energetic point of view SNRs are viable sources of Galactic CRs, the issue of whether they can accelerate protons up to PeV remains unsolved. Here we discuss particle acceleration at the forward shock of SN and discuss the possibility that the escaping particle current may excite a non-resonant instability that in turn leads to the formation of resonant modes confining particles close to the shock and increasing the maximum energy. This mechanism works throughout the expansion of the SN explosion, from the ejecta dominated (ED to the Sedov-Taylor (ST phase. Because of their higher explosion rate,we focus on type II SNae expanding in the slow, dense red supergiant wind. When the explosion occurs in such winds, the transition between the ED and the ST phase is likely to take place within a few tens of years. As a result, the spectrum of accelerated particles shows a break in the slope, at the maximum energy (EM achieved at the beginning of the ST phase. Above this energy, the spectrum becomes steeper but remains a power law than developing an exponential cutoff. We show that for type II SNae typical parameters, proton EM can easily reach PeV energies, confirming that type II SNRs are the best candidate sources for CRs at the knee. We have tried to fit KASCADE-Grande, ARGO -YBJ and YAC1-Tibet Array data with our model but we could not find any parameter combination that could explain all data sets. Indeed the recent measurement of the proton and helium spectra in the knee region, with the ARGO-YBJ and YAC1-Tibet Array, has made the situation very confused. These measurements suggest that the knee in the light component is at 650 TeV, appreciably below the overall spectrum knee. This finding would resolve the problem of reaching very high energies in SNae, but, on the other hand, it would open a critical issue in the transition region between Galactic and extragalactic CRs.

  4. Expanding the spectrum of human ganglionic eminence region anomalies on fetal magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Righini, Andrea; Parazzini, Cecilia; Izzo, Giana [Children' s Hospital ' ' V. Buzzi' ' , Department of Radiology and Neuroradiology, Milan (Italy); Cesaretti, Claudia [Children' s Hospital ' ' V. Buzzi' ' , Department of Radiology and Neuroradiology, Milan (Italy); Ospedale Maggiore Policlinico, Medical Genetics Unit, Fondazione I.R.C.C.S. Ca' Granda, Milan (Italy); Conte, Giorgio [Children' s Hospital ' ' V. Buzzi' ' , Department of Radiology and Neuroradiology, Milan (Italy); University of Milan, Department of Health Sciences, Milan (Italy); Frassoni, Carolina; Inverardi, Francesca [Fondazione I.R.C.C.S. Istituto Neurologico ' ' C. Besta' ' , Clinical Epileptology and Experimental Neurophysiology Unit, Milan (Italy); Bulfamante, Gaetano; Avagliano, Laura [San Paolo Hospital, Division of Human Pathology, Milan (Italy); Rustico, Mariangela [Children' s Hospital ' ' V. Buzzi' ' , Department of Obstetrics and Gynaecology, Prenatal Diagnosis, Milan (Italy)

    2016-03-15

    Ganglionic eminence (GE) is a transient fetal brain structure that harvests a significant amount of precursors of cortical GABA-ergic interneurons. Prenatal magnetic resonance (MR) imaging features of GE anomalies (i.e., cavitations) have already been reported associated with severe micro-lissencephaly. The purpose of this report was to illustrate the MR imaging features of GE anomalies in conditions other than severe micro-lissencephalies. Among all the fetuses submitted to prenatal MR imaging at our center from 2005 to 2014, we collected eight cases with GE anomalies and only limited associated brain anomalies. The median gestational age at the time of MR imaging was 21 weeks ranging from 19 to 29 weeks. Two senior pediatric neuroradiologists categorized the anomalies of the GE region in two groups: group one showing cavitation in the GE region and group two showing enlarged GE region. For each fetal case, associated cranial anomalies were also reported. Five out of the eight cases were included in group one and three in group two. Besides the GE region abnormality, all eight cases had additional intracranial anomalies, such as mild partial callosal agenesis, vermian hypoplasia and rotation, cerebellar hypoplasia, ventriculomegaly, enlarged subarachnoid spaces, molar tooth malformation. Ultrasound generally detected most of the associated intracranial anomalies, prompting the MR investigation; on the contrary in none of the cases, GE anomalies had been detected by ultrasound. Our observation expands the spectrum of human GE anomalies, demonstrating that these may take place also without associated severe micro-lissencephalies. (orig.)

  5. Brainstem reflexes in patients with familial dysautonomia.

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    Gutiérrez, Joel V; Norcliffe-Kaufmann, Lucy; Kaufmann, Horacio

    2015-03-01

    Several distinctive clinical features of patients with familial dysautonomia (FD) including dysarthria and dysphagia suggest a developmental defect in brainstem reflexes. Our aim was to characterize the neurophysiological profile of brainstem reflexes in these patients. We studied the function of sensory and motor trigeminal tracts in 28 patients with FD. All were homozygous for the common mutation in the IKAP gene. Each underwent a battery of electrophysiological tests including; blink reflexes, jaw jerk reflex, masseter silent periods and direct stimulation of the facial nerve. Responses were compared with 25 age-matched healthy controls. All patients had significantly prolonged latencies and decreased amplitudes of all examined brainstem reflexes. Similar abnormalities were seen in the early and late components. In contrast, direct stimulation of the facial nerve revealed relative preservation of motor responses. The brainstem reflex abnormalities in FD are best explained by impairment of the afferent and central pathways. A reduction in the number and/or excitability of trigeminal sensory axons is likely the main problem. These findings add further evidence to the concept that congenital mutations of the elongator-1 protein (or IKAP) affect the development of afferent neurons including those carrying information for the brainstem reflex pathways. Copyright © 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  6. Probing the Scale Invariance of the Inflationary Power Spectrum in Expanding Quasi-Two-Dimensional Dipolar Condensates

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    Chä, Seok-Yeong; Fischer, Uwe R.

    2017-03-01

    We consider an analogue de Sitter cosmos in an expanding quasi-two-dimensional Bose-Einstein condensate with dominant dipole-dipole interactions between the atoms or molecules in the ultracold gas. It is demonstrated that a hallmark signature of inflationary cosmology, the scale invariance of the power spectrum of inflaton field correlations, experiences strong modifications when, at the initial stage of expansion, the excitation spectrum displays a roton minimum. Dipolar quantum gases thus furnish a viable laboratory tool to experimentally investigate, with well-defined and controllable initial conditions, whether primordial oscillation spectra deviating from Lorentz invariance at trans-Planckian momenta violate standard predictions of inflationary cosmology.

  7. Dysautonomias in Amyloidosis: : Need for an interdisciplinary approach

    NARCIS (Netherlands)

    Hazenberg, B. P. C.

    2013-01-01

    Systemic amyloidosis is a life-threatening and frequently unrecognized cause of dysautonomia. Autonomic neuropathy is a common manifestation of AL amyloidosis (caused by deposition of an immunoglobulin free light chain produced by an underlying plasma cell clone) and of autosomal dominant hereditary

  8. Dental management of a child with familial dysautonomia.

    Science.gov (United States)

    Nussbaum, B L

    1986-01-01

    Familial dysautonomia is a hereditary sensory neuropathy that involves sensory, motor, and central components of the nervous system. Orofacial features include a tendency toward facial concavity in the child, and convexity in the adult. There is increased salivation, and crowded teeth and malocclusion are characteristic. This patient is not Jewish; this makes his case an extremely rare one.

  9. Contemporary perioperative management of adult familial dysautonomia (Riley-Day syndrome).

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    Milne, Andrew; Mon, Wint Yu; Down, James; Obichere, Austin; Ackland, Gareth L

    2015-05-01

    Familial dysautonomia (Riley-Day syndrome) is a rare multisystem disorder associated with an excess risk of perioperative morbidity and mortality. Because life expectancy is limited, few reports consider the perioperative management of familial dysautonomia in adults with advanced disease and end-organ dysfunction. Here, we report on the management of an adult patient with familial dysautonomia, highlighting recent developments in perioperative technology and pharmacology of special relevance to this challenging population.

  10. Cerebral autoregulation and symptoms of orthostatic hypotension in familial dysautonomia.

    Science.gov (United States)

    Fuente Mora, Cristina; Palma, Jose-Alberto; Kaufmann, Horacio; Norcliffe-Kaufmann, Lucy

    2017-07-01

    Familial dysautonomia is an inherited autonomic disorder with afferent baroreflex failure. We questioned why despite low blood pressure standing, surprisingly few familial dysautonomia patients complain of symptomatic hypotension or have syncope. Using transcranial Doppler ultrasonography of the middle cerebral artery, we measured flow velocity (mean, peak systolic, and diastolic), area under the curve, pulsatility index, and height of the dictrotic notch in 25 patients with familial dysautonomia and 15 controls. In patients, changing from sitting to a standing position, decreased BP from 124 ± 4/64 ± 3 to 82 ± 3/37 ± 2 mmHg (p < 0.0001, for both). Despite low BP, all patients denied orthostatic symptoms. Middle cerebral artery velocity fell minimally, and the magnitude of the reductions were similar to those observed in healthy controls, in whom BP upright did not fall. While standing, patients had a greater fall in cerebrovascular resistance (p < 0.0001), an increase in pulsatility (p < 0.0001), and a deepening of the dicrotic notch (p = 0.0010), findings all consistent with low cerebrovascular resistance. No significant changes occurred in controls. Patients born with baroreflex deafferentation retain the ability to buffer wide fluctuations in BP and auto-regulate cerebral blood flow. This explains how they can tolerate extremely low BPs standing that would otherwise induce syncope.

  11. New ALS-Related Genes Expand the Spectrum Paradigm of Amyotrophic Lateral Sclerosis.

    Science.gov (United States)

    Sabatelli, Mario; Marangi, Giuseppe; Conte, Amelia; Tasca, Giorgio; Zollino, Marcella; Lattante, Serena

    2016-03-01

    Amyotrophic Lateral Sclerosis (ALS) is characterized by the degeneration of upper and lower motor neurons. Clinical heterogeneity is a well-recognized feature of the disease as age of onset, site of onset and the duration of the disease can vary greatly among patients. A number of genes have been identified and associated to familial and sporadic forms of ALS but the majority of cases remains still unexplained. Recent breakthrough discoveries have demonstrated that clinical manifestations associated with ALS-related genes are not circumscribed to motor neurons involvement. In this view, ALS appears to be linked to different conditions over a continuum or spectrum in which overlapping phenotypes may be identified. In this review, we aim to examine the increasing number of spectra, including ALS/Frontotemporal Dementia and ALS/Myopathies spectra. Considering all these neurodegenerative disorders as different phenotypes of the same spectrum can help to identify common pathological pathways and consequently new therapeutic targets in these incurable diseases.

  12. Gaze palsy, hypogeusia and a probable association with miscarriage of pregnancy--the expanding clinical spectrum of non-opticospinal neuromyelitis optica spectrum disorders: a case report.

    Science.gov (United States)

    Chang, Thashi; Withana, Milinda

    2015-02-10

    serum. Her vomiting subsided with immunosuppressive therapy. Her second pregnancy had resulted in a first-trimester miscarriage. The clinical spectrum of neuromyelitis optica spectrum disorders has expanded beyond optic neuritis and myelitis to include non-opticospinal syndromes involving the diencephalon, brainstem and cerebrum. Our report highlights the varied central nervous system manifestations of neuromyelitis optica spectrum disorders and miscarriage of pregnancy possibly related to anti-aquaporin-4 antibodies.

  13. Neonatal Screening: Some Ethical Issues of Expanding Spectrum for Genetically Determined Diseases

    Directory of Open Access Journals (Sweden)

    S. S. Deryabina

    2015-01-01

    Full Text Available The article considers philosophical questions of neonatal screening technology. The main focus is on ethical and methodological issues that inevitably arise when expanding the number of scanned nosologies and applying genetic research methods. Questions concerning the existing discrepancy between technical capacity and the practical level of healthcare delivery and the probabilistic nature of results obtained by molecular testing are analyzed in terms of methodology. Access to information about the DNA-testing of newborns and the linkage between neonatal screening and prenatal diagnostics are among the most topical ethical problems raised within this article. One of the purposes of this article is to draw the attention of the public — especially it concerns current and prospective parents and volunteer organizations — to these contemporary problems.

  14. The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies

    Directory of Open Access Journals (Sweden)

    Sarosh R Irani

    2012-04-01

    Full Text Available The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1, CASPR2 (contactin-associated protein 2, and contactin-2, and NMDA (N-methyl, D-aspartate -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.

  15. Expanding the genotype-phenotype spectrum in hereditary colorectal cancer by gene panel testing

    DEFF Research Database (Denmark)

    Rohlin, Anna; Rambech, Eva; Kvist, Anders;

    2016-01-01

    sixteen pathogenic or likely pathogenic variants and 30 variants of unknown clinical significance. Four of the pathogenic or likely pathogenic variants were found in BMPR1A in patients with unexplained familial adenomatous polyposis or atypical adenomatous polyposis, which extends the genotype......-phenotype spectrum for this gene. Nine patients had more than one variant remaining after the filtration, including three with truncating mutations in BMPR1A, PMS2 and AXIN2. CNVs were found in three patients, in upstream regions of SMAD4, MSH3 and CTNNB1, and one additional individual harbored a 24.2 kb duplication......Hereditary syndromes causing colorectal cancer include both polyposis and non-polyposis syndromes. Overlapping phenotypes between the syndromes have been recognized and this make targeted molecular testing for single genes less favorable, instead there is a gaining interest for multi-gene panel...

  16. Law as a Tool for Preventing Chronic Diseases: Expanding the Spectrum of Effective Public Health Strategies

    Directory of Open Access Journals (Sweden)

    George A. Mensah

    2004-04-01

    Full Text Available In part one of this 2-part series, we reviewed the important roles that laws have played in public health and provided examples of specific laws and their effectiveness in supporting public health interventions (1. We suggested that conceptual legal frameworks for systematically applying law to preventing and controlling chronic diseases have not been fully recognized and we provided the basic elements of a conceptual legal framework. In part 2 of this series, we first provide an overview of U.S. jurisprudence, describe the legal mechanisms, remedies, and tools for applying law to public health, and summarize the jurisdictional levels at which laws, mechanisms, remedies, and tools operate. We then identify the potential contours for legal frameworks of varying complexity and scope by offering examples of legal frameworks in public health practice. This paper also outlines a plan for increasing the capacity within the Centers for Disease Control and Prevention (CDC for developing legal frameworks and expanding guidance on using legal tools for preventing and controlling chronic diseases. Finally, we describe resources for building or enhancing the capacity to use law as a tool for preventing diseases, injuries, and disabilities at the local level.

  17. Providing specialty consultant expertise to primary care: an expanding spectrum of modalities.

    Science.gov (United States)

    Kirsh, Susan R; Ho, P Michael; Aron, David C

    2014-10-01

    In most models of health care delivery, the bulk of services are provided in primary care and there is frequent request for the input of specialty consultants. A critical issue for current and future health care systems is the effective and efficient delivery of specialist expertise for clinicians and patients. Input on a patient's care from specialty consultants usually requires a face-to-face visit between the patient and the consultant. New and complementary models of knowledge sharing have emerged. We describe a framework assessment of a spectrum of knowledge-sharing methods in the context of a patient-centered medical home. This framework is based on our experience in the Veterans Health Administration and a purposive review of the literature. These newer modes of specialty consultation include electronic consultation, secure text messaging, telemedicine of various types, and population preemptive consults. In addition to describing these modes of consultation, our framework points to several important areas in which further research is needed to optimize effectiveness.

  18. Molecular analysis expands the spectrum of phenotypes associated with GLI3 mutations

    Science.gov (United States)

    Johnston, Jennifer J.; Sapp, Julie C.; Turner, Joyce T.; Amor, David; Aftimos, Salim; Aleck, Kyrieckos A.; Bocian, Maureen; Bodurtha, Joann N.; Cox, Gerald F.; Curry, Cynthia J.; Day, Ruth; Donnai, Dian; Field, Michael; Fujiwara, Ikuma; Gabbett, Michael; Gal, Moran; Graham, John M.; Hedera, Peter; Hennekam, Raoul C.M.; Hersh, Joseph H.; Hopkin, Robert J.; Kayserili, Hülya; Kidd, Alexa M.J.; Kimonis, Virginia; Lin, Angela E.; Lynch, Sally Ann; Maisenbacher, Melissa; Mansour, Sahar; McGaughran, Julie; Mehta, Lakshmi; Murphy, Helen; Raygada, Margarita; Robin, Nathaniel H.; Rope, Alan F.; Rosenbaum, Kenneth N.; Schaefer, G. Bradley; Shealy, Amy; Smith, Wendy; Soller, Maria; Sommer, Annmarie; Stalker, Heather J.; Steiner, Bernhard; Stephan, Mark J.; Tilstra, David; Tomkins, Susan; Trapane, Pamela; Tsai, Anne Chun-Hui; Van Allen, Margot I.; Vasudevan, Pradeep C.; Zabel, Bernhard; Zunich, Janice; Black, Graeme C.M.; Biesecker, Leslie G.

    2010-01-01

    A range of phenotypes including Greig cephalopolysyndactyly and Pallister-Hall syndromes (GCPS, PHS) are caused by pathogenic mutation of the GLI3 gene. To characterize the clinical variability of GLI3 mutations, we present a subset of a cohort of 174 probands referred for GLI3 analysis. Eighty-one probands with typical GCPS or PHS were previously reported, and we report the remaining ninety-three probands here. This includes nineteen probands (twelve mutations) who fulfilled clinical criteria for GCPS or PHS, forty-eight probands (sixteen mutations) with features of GCPS or PHS but who did not meet the clinical criteria (sub-GCPS and sub-PHS), twenty-one probands (six mutations) with features of PHS or GCPS and oral-facial-digital syndrome and five probands (one mutation) with non-syndromic polydactyly. These data support previously identified genotype-phenotype correlations and demonstrate a more variable degree of severity than previously recognized. The finding of GLI3 mutations in patients with features of oral-facial-digital syndrome supports the observation that GLI3 interacts with cilia. We conclude that the phenotypic spectrum of GLI3 mutations is broader than that encompassed by the clinical diagnostic criteria, but the phenotype-genotype correlation persists. Individuals with features of either GCPS or PHS should be screened for mutations in GLI3 even if they do not fulfill clinical criteria. PMID:20672375

  19. Familial dysautonomia (Riley-Day syndrome): when baroreceptor feedback fails.

    Science.gov (United States)

    Norcliffe-Kaufmann, Lucy; Kaufmann, Horacio

    2012-12-24

    Familial dysautonomia (FD) is a rare hereditary disorder caused by mutations within the gene that encodes for I-κ-B kinase complex associated protein (IKAP). A deficiency of IKAP affects the development of primary sensory neurons including those carrying baroreflex afferent volleys, a feature that explains their characteristic sensory loss and labile blood pressure. This review describes the history, the genotype of FD and the unusual cardiovascular autonomic phenotype of these patients. We outline the main consequences of a failure to receive information from arterial baroreceptors, including the characteristic "autonomic storms" and severe end-organ target damage.

  20. Spine deformity in familial dysautonomia (Riley-Day syndrome).

    Science.gov (United States)

    Albanese, S A; Bobechko, W P

    1987-01-01

    Familial dysautonomia spanning a 30-year period reviewed retrospectively. The 16 patients identified included nine with spine deformities. Serious general medical problems were common. Spine deformities included kyphosis, scoliosis, or a combination of both. Brace treatment was attempted and was unsuccessful in three patients. Seven underwent surgical stabilization. Although all seven patients tolerated the surgical procedures, at least one postoperative complication was noted in each case. Follow-up ranged from 1 1/2 to 16 1/2 years. We recommend close observation of the deformities, early stabilization when disorders are progressive, and a high level of awareness of potential complications.

  1. Expanding the spectrum of congenital anomalies of the diencephalic-mesencephalic junction

    Energy Technology Data Exchange (ETDEWEB)

    Severino, Mariasavina; Tortora, Domenico; Rossi, Andrea [Istituto Giannina Gaslini, Neuroradiology Unit, Genova (Italy); Pistorio, Angela [Istituto Giannina Gaslini, Epidemiology and Biostatistics Unit, Genoa (Italy); Ramenghi, Luca Antonio [Istituto Giannina Gaslini, Neonatal Intensive Care Unit, Genoa (Italy); Napoli, Flavia [Istituto Giannina Gaslini, Endocrinology Unit, Genoa (Italy); Mancardi, Maria Margherita [Istituto Giannina Gaslini, Neuropsychiatry Unit, Genoa (Italy); Striano, Pasquale [Istituto Giannina Gaslini, Paediatric Neurology and Muscular Diseases Unit, Genoa (Italy); Capra, Valeria [Istituto Giannina Gaslini, Genetic Unit, Genoa (Italy)

    2016-01-15

    We aimed to describe the clinico-radiological findings of patients with disorders of diencephalic-mesencephalic junction (DMJ) formation and midbrain anteroposterior patterning. We reviewed the DMJ anatomy of 445 patients with brain malformations. Associated supra/infratentorial abnormalities and clinical findings were noted. Craniocaudal and anteroposterior diameters of midbrain, pons, medulla, vermis, and transverse cerebellar diameter were compared with age-matched controls. Post hoc tests were corrected according to Bonferroni (p{sub B}). Two patterns of DMJ anomaly were identified in 12 patients (7 females, mean age 41 months). Type A was characterized by hypothalamic-mesencephalic fusion on axial plane, with possible midbrain ventral cleft (7 patients). Anteroposterior (p{sub B} =.006) and craniocaudal (p{sub B} =.027) diameters of the pons, craniocaudal diameter of the vermis (p{sub B} =.015), and transverse cerebellar diameter (p{sub B} =.011) were smaller than the controls. Corticospinal tract, basal ganglia, and commissural anomalies were also associated. Clinical findings included spastic-dystonic tetraparesis, hypothalamic dysfunction, epilepsy, and severe developmental delay. Type B was characterized by incomplete thalamic-mesencephalic cleavage on sagittal plane, with parenchymal bands connecting the interthalamic adhesion with the midbrain (five patients). Anteroposterior diameters of midbrain (p{sub B} =.002), pons (p{sub B} =.0004), and medulla (p{sub B} =.002) as well as the vermian anteroposterior (p{sub B} =.040) and craniocaudal diameters (p{sub B} =.014) were smaller than the controls. These patients were less neurologically impaired, most presenting mild developmental delay. The spectrum of DMJ patterning defects is wide and may be associated with several brain malformations. Infratentorial brain structures should be carefully evaluated to better define the type of associated midbrain-hindbrain anomalies. (orig.)

  2. Prenatal growth restriction, retinal dystrophy, diabetes insipidus and white matter disease: expanding the spectrum of PRPS1-related disorders.

    Science.gov (United States)

    Al-Maawali, Almundher; Dupuis, Lucie; Blaser, Susan; Heon, Elise; Tarnopolsky, Mark; Al-Murshedi, Fathiya; Marshall, Christian R; Paton, Tara; Scherer, Stephen W; Roelofsen, Jeroen; van Kuilenburg, André B P; Mendoza-Londono, Roberto

    2015-03-01

    PRPS1 codes for the enzyme phosphoribosyl pyrophosphate synthetase-1 (PRS-1). The spectrum of PRPS1-related disorders associated with reduced activity includes Arts syndrome, Charcot-Marie-Tooth disease-5 (CMTX5) and X-linked non-syndromic sensorineural deafness (DFN2). We describe a novel phenotype associated with decreased PRS-1 function in two affected male siblings. Using whole exome and Sanger sequencing techniques, we identified a novel missense mutation in PRPS1. The clinical phenotype in our patients is characterized by high prenatal maternal α-fetoprotein, intrauterine growth restriction, dysmorphic facial features, severe intellectual disability and spastic quadraparesis. Additional phenotypic features include macular coloboma-like lesions with retinal dystrophy, severe short stature and diabetes insipidus. Exome sequencing of the two affected male siblings identified a shared putative pathogenic mutation c.586C>T p.(Arg196Trp) in the PRPS1 gene that was maternally inherited. Follow-up testing showed normal levels of hypoxanthine in urine samples and uric acid levels in blood serum. The PRS activity was significantly reduced in erythrocytes of the two patients. Nucleotide analysis in erythrocytes revealed abnormally low guanosine triphosphate and guanosine diphosphate. This presentation is the most severe form of PRPS1-deficiency syndrome described to date and expands the spectrum of PRPS1-related disorders.

  3. Screening of ARHSP-TCC patients expands the spectrum of SPG11 mutations and includes a large scale gene deletion.

    Science.gov (United States)

    Denora, Paola S; Schlesinger, David; Casali, Carlo; Kok, Fernando; Tessa, Alessandra; Boukhris, Amir; Azzedine, Hamid; Dotti, Maria Teresa; Bruno, Claudio; Truchetto, Jeremy; Biancheri, Roberta; Fedirko, Estelle; Di Rocco, Maja; Bueno, Clarissa; Malandrini, Alessandro; Battini, Roberta; Sickl, Elisabeth; de Leva, Maria Fulvia; Boespflug-Tanguy, Odile; Silvestri, Gabriella; Simonati, Alessandro; Said, Edith; Ferbert, Andreas; Criscuolo, Chiara; Heinimann, Karl; Modoni, Anna; Weber, Peter; Palmeri, Silvia; Plasilova, Martina; Pauri, Flavia; Cassandrini, Denise; Battisti, Carla; Pini, Antonella; Tosetti, Michela; Hauser, Erwin; Masciullo, Marcella; Di Fabio, Roberto; Piccolo, Francesca; Denis, Elodie; Cioni, Giovanni; Massa, Roberto; Della Giustina, Elvio; Calabrese, Olga; Melone, Marina A B; De Michele, Giuseppe; Federico, Antonio; Bertini, Enrico; Durr, Alexandra; Brockmann, Knut; van der Knaap, Marjo S; Zatz, Mayana; Filla, Alessandro; Brice, Alexis; Stevanin, Giovanni; Santorelli, Filippo M

    2009-03-01

    Autosomal recessive spastic paraplegia with thinning of corpus callosum (ARHSP-TCC) is a complex form of HSP initially described in Japan but subsequently reported to have a worldwide distribution with a particular high frequency in multiple families from the Mediterranean basin. We recently showed that ARHSP-TCC is commonly associated with mutations in SPG11/KIAA1840 on chromosome 15q. We have now screened a collection of new patients mainly originating from Italy and Brazil, in order to further ascertain the spectrum of mutations in SPG11, enlarge the ethnic origin of SPG11 patients, determine the relative frequency at the level of single Countries (i.e., Italy), and establish whether there is one or more common mutation. In 25 index cases we identified 32 mutations; 22 are novel, including 9 nonsense, 3 small deletions, 4 insertions, 1 in/del, 1 small duplication, 1 missense, 2 splice-site, and for the first time a large genomic rearrangement. This brings the total number of SPG11 mutated patients in the SPATAX collection to 111 cases in 44 families and in 17 isolated cases, from 16 Countries, all assessed using homogeneous clinical criteria. While expanding the spectrum of mutations in SPG11, this larger series also corroborated the notion that even within apparently homogeneous population a molecular diagnosis cannot be achieved without full gene sequencing.

  4. Autism in the Son of a Woman with Mitochondrial Myopathy and Dysautonomia: A Case Report.

    Science.gov (United States)

    Brown, Bradley D; Rais, Theodore

    2015-01-01

    The relationship between autism spectrum disorders and mitochondrial dysfunction, including mitochondrial myopathies and other mitochondrial diseases, is an area of ongoing research. All autism spectrum disorders are known to be heritable, via genetic and/or epigenetic mechanisms, but specific modes of inheritance are not well characterized. Nevertheless, autism spectrum disorders have been linked to many specific genes associated with mitochondrial function, especially to genes involved in mitochondrial tRNA and the electron transport chain, both particularly vulnerable to point mutations, and clinical research also supports a relationship between the two pathologies. Although only a small minority of patients with autism have a mitochondrial disease, many patients with mitochondrial myopathies have autism spectrum disorder symptoms, and these symptoms may be the presenting symptoms, which presents a diagnostic challenge for clinicians. The authors report the case of a 15-year-old boy with a history of autism spectrum disorder and neurocardiogenic syncope, admitted to the inpatient unit for self-injury, whose young mother, age 35, was discovered to suffer from mitochondrial myopathy, dysautonomia, neurocardiogenic syncope, Ehler-Danlos syndrome, and other uncommon multisystem pathologies likely related to mitochondrial dysfunction. This case illustrates the need for a high index of suspicion for mitochondrial disease in patients with autism, as they have two orders of magnitude greater risk for such diseases than the general population. The literature shows that mitochondrial disease is underdiagnosed in autism spectrum disorder patients and should not be viewed as a "zebra" (i.e., an obscure diagnosis that is made when a more common explanation is more likely).

  5. Expanding the Spectrum of Genes Involved in Huntington Disease Using a Combined Clinical and Genetic Approach.

    Science.gov (United States)

    Mariani, Louise-Laure; Tesson, Christelle; Charles, Perrine; Cazeneuve, Cécile; Hahn, Valérie; Youssov, Katia; Freeman, Leorah; Grabli, David; Roze, Emmanuel; Noël, Sandrine; Peuvion, Jean-Noel; Bachoud-Levi, Anne-Catherine; Brice, Alexis; Stevanin, Giovanni; Durr, Alexandra

    2016-09-01

    Huntington disease (HD), a prototypic monogenic disease, is caused by an expanded CAG repeat in the HTT gene exceeding 35 units. However, not all patients with an HD phenotype carry the pathological expansion in HTT, and the positive diagnosis rate is poor. To examine patients with HD phenotypes to determine the frequency of HD phenocopies with typical features of HD but without pathological CAG repeat expansions in HTT in an attempt to improve the positive diagnosis rate. Between January 1, 2004, and April 18, 2011, a total of 226 consecutive index patients with an HD phenotype were referred to specialized clinics of the French National Huntington Disease Reference Centre for Rare Diseases. They underwent detailed clinical examination and follow-up, as well as neuropsychological, biological, imaging, and genetic examinations. Nucleotide expansions in JPH3, ATN1, TBP, and C9ORF72 and mutations in PRNP, as well as acquired conditions commonly causing HD phenocopies, were first screened. The diagnostic rate of HD phenocopies and frequency of other etiologies using deep clinical phenotyping and next generation sequencing. Our goal was to improve the genetic diagnosis of HD phenocopies and to identify new HD related genes. One hundred ninety-eight patients carried a pathological CAG repeat expansion in HTT, whereas 28 patients (12 women and 16 men) did not. Huntington disease phenocopies accounted for 12.4%, and their mean (SD) age at onset was similar to those of the HD-HTT group (47.3 [12.7] years vs 50.3 [16.4] years, P = .29). We first identified 3 patients with abnormal CTG expansions in JPH3, a fourth patient with an antiphospholipid syndrome, and a fifth patient with B12 avitaminosis. A custom-made 63-gene panel was generated based on clinical evolution and exome sequencing. It contained genes responsible for HD phenocopies and other neurodegenerative conditions, as well as candidate genes from exome sequencing in 3 index cases with imaging features of brain

  6. Dysautonomia, a Heuristic Approach to a Revised Model for Etiology of Disease

    Directory of Open Access Journals (Sweden)

    Derrick Lonsdale

    2009-01-01

    Full Text Available Dysautonomia refers to a disease where the autonomic nervous system is dysfunctional. This may be a central control mechanism, as in genetically determined familial dysautonomia (Riley-Day Syndrome, or peripherally in the distribution of the sympathetic and parasympathetic systems. There are multiple reports of a number of different diseases associated with dysautonomia. The etiology of this association has never been explained. There are also multiple publications on dysautonomia associated with specific non-caloric nutritional deficiencies. Beriberi is the prototype of autonomic dysfunction. It is the best known nutritional deficiency disease caused by an imbalance between ingested calories and the vitamins required for their oxidation, particularly thiamin. Long thought to be abolished in modern medical thinking, there are occasional isolated reports of the full-blown disease in developed Western cultures.

  7. Cardiac‐locked bursts of muscle sympathetic nerve activity are absent in familial dysautonomia

    National Research Council Canada - National Science Library

    Macefield, Vaughan G; Norcliffe‐Kaufmann, Lucy; Axelrod, Felicia B; Kaufmann, Horacio

    2013-01-01

    •  Patients with familial dysautonomia cannot regulate their blood pressure: on being tilted upright, their blood pressure and heart rate fall, but both can rise to high levels during emotional arousal...

  8. Reversible parasympathetic dysautonomia following stinging attributed to the box jelly fish (Chironex fleckeri).

    Science.gov (United States)

    Chand, R P; Selliah, K

    1984-10-01

    Following a box jelly fish sting, a 52 year old Chinese fisherman developed acute abdominal distension, inability to pass urine and failure of erection. Examination revealed gaseous abdominal distension and a distended urinary bladder. Absence of lachrimation and absence of changes in the R-R interval in the ECG during breathing and carotid sinus massage gave further evidence of parasympathetic dysautonomia. The patient made a complete recovery. The case highlights the occurrence of reversible parasympathetic dysautonomia following box jelly fish sting.

  9. Nonspinal orthopaedic problems in familial dysautonomia (Riley-Day syndrome).

    Science.gov (United States)

    Laplaza, F J; Turajane, T; Axelrod, F B; Burke, S W

    2001-01-01

    Familial dysautonomia (FD) is a rare autosomal recessive disease occurring in Ashkenazi Jews. It affects the autonomic, central, and peripheral nervous systems. The purpose of this study was to assess the prevalence and characteristics of orthopedic deformities, other than spinal deformities, in this population. A retrospective review of the medical records and radiographs of 182 patients was made. Three main groups of orthopaedic conditions were evaluated: (a) Fractures: 60% of the patients had one or more fractures; the average fracture rate was 1.4/patient. (b) Neuropathic joints: 11% of the cases had one or more neuropathic joints, the knee being the most common. (c) Other musculoskeletal deformities: 26% of the patients had one or more deformities. Lower extremity rotational problems and foot anomalies accounted for most of these deformities. Patients with FD have a higher prevalence of fractures and neuropathic joints than do their peers. The fracture pattern also is different, with a higher incidence of proximal femoral fractures.

  10. Evaluation of congenital dysautonomia other than Riley-Day syndrome.

    Science.gov (United States)

    Alvarez, E; Ferrer, T; Pérez-Conde, C; López-Terradas, J M; Pérez-Jiménez, A; Ramos, M J

    1996-02-01

    We report on four children, from different families, who suffer from a congenital autonomic disorder, presumably inherited. Three of them have a sensory neuropathy but do not fit any described hereditary sensory and autonomic neuropathy. All four were examined along with some of their immediate family members. We assessed the cardiovagal, sympathetic adrenergic and sympathetic cholinergic functions with a battery of non-invasive tests. Results demonstrated that sudomotor and cardiovascular orthostatic regulation exhibited the greatest abnormalities, pointing to a predominant impairment of sympathetic components, both cholinergic and adrenergic. The overall examination showed a heterogeneous group of congenital dysautonomia, exclusive of Riley-Day or other recognized hereditary sensory and autonomic neuropathies. We emphasize the importance of studying whole family groups to diagnose subclinical impairment and to provide correct genetic counselling.

  11. Twelve positions in a β-lactamase that can expand its substrate spectrum with a single amino acid substitution.

    Directory of Open Access Journals (Sweden)

    Hyojeong Yi

    Full Text Available The continuous evolution of β-lactamases resulting in bacterial resistance to β-lactam antibiotics is a major concern in public health, and yet the underlying molecular basis or the pattern of such evolution is largely unknown. We investigated the mechanics of the substrate fspectrum expansion of the class A β-lactamase using PenA of Burkholderia thailandensis as a model. By analyzing 516 mutated enzymes that acquired the ceftazidime-hydrolyzing activity, we found twelve positions with single amino acid substitutions (altogether twenty-nine different substitutions, co-localized at the active-site pocket area. The ceftazidime MIC (minimum inhibitory concentration levels and the relative frequency in the occurrence of substitutions did not correlate well with each other, and the latter appeared be largely influenced by the intrinsic mutational biases present in bacteria. Simulation studies suggested that all substitutions caused a congruent effect, expanding the space in a conserved structure called the omega loop, which in turn increased flexibility at the active site. A second phase of selection, in which the mutants were placed under increased antibiotic pressure, did not result in a second mutation in the coding region, but a mutation that increased gene expression arose in the promoter. This result suggests that the twelve amino acid positions and their specific substitutions in PenA may represent a comprehensive repertoire of the enzyme's adaptability to a new substrate. These mapped substitutions represent a comprehensive set of general mechanical paths to substrate spectrum expansion in class A β-lactamases that all share a functional evolutionary mechanism using common conserved residues.

  12. Comprehensive genotyping and clinical characterisation reveal 27 novel NKX2-1 mutations and expand the phenotypic spectrum

    Science.gov (United States)

    Thorwarth, Anne; Schnittert-Hübener, Sarah; Schrumpf, Pamela; Müller, Ines; Jyrch, Sabine; Dame, Christof; Biebermann, Heike; Kleinau, Gunnar; Katchanov, Juri; Schuelke, Markus; Ebert, Grit; Steininger, Anne; Bönnemann, Carsten; Brockmann, Knut; Christen, Hans-Jürgen; Crock, Patricia; deZegher, Francis; Griese, Matthias; Hewitt, Jacqueline; Ivarsson, Sten; Hübner, Christoph; Kapelari, Klaus; Plecko, Barbara; Rating, Dietz; Stoeva, Iva; Ropers, Hans-Hilger; Grüters, Annette; Ullmann, Reinhard; Krude, Heiko

    2017-01-01

    Background NKX2-1 encodes a transcription factor with large impact on the development of brain, lung and thyroid. Germline mutations of NKX2-1 can lead to dysfunction and malformations of these organs. Starting from the largest coherent collection of patients with a suspected phenotype to date, we systematically evaluated frequency, quality and spectrum of phenotypic consequences of NKX2-1 mutations. Methods After identifying mutations by Sanger sequencing and array CGH, we comprehensively reanalysed the phenotype of affected patients and their relatives. We employed electrophoretic mobility shift assay (EMSA) to detect alterations of NKX2-1 DNA binding. Gene expression was monitored by means of in situ hybridisation and compared with the expression level of MBIP, a candidate gene presumably involved in the disorders and closely located in close genomic proximity to NKX2-1. Results Within 101 index patients, we detected 17 point mutations and 10 deletions. Neurological symptoms were the most consistent finding (100%), followed by lung affection (78%) and thyroidal dysfunction (75%). Novel symptoms associated with NKX2-1 mutations comprise abnormal height, bouts of fever and cardiac septum defects. In contrast to previous reports, our data suggest that missense mutations in the homeodomain of NKX2-1 not necessarily modify its DNA binding capacity and that this specific type of mutations may be associated with mild pulmonary phenotypes such as asthma. Two deletions did not include NKX2-1, but MBIP, whose expression spatially and temporarily coincides with NKX2-1 in early murine development. Conclusions The high incidence of NKX2-1 mutations strongly recommends the routine screen for mutations in patients with corresponding symptoms. However, this analysis should not be confined to the exonic sequence alone, but should take advantage of affordable NGS technology to expand the target to adjacent regulatory sequences and the NKX2-1 interactome in order to maximise the

  13. Melanotic MiT family translocation neoplasms: Expanding the clinical and molecular spectrum of this unique entity of tumors.

    Science.gov (United States)

    Saleeb, Rola M; Srigley, John R; Sweet, Joan; Doucet, Cedric; Royal, Virginie; Chen, Ying-Bei; Brimo, Fadi; Evans, Andrew

    2017-08-25

    MiT family translocation tumors are a group of neoplasms characterized by translocations involving MiT family transcription factors. The translocation renal cell carcinomas, TFE3 (Xp11.2) and TFEB (t6;11) are known members of this family. Melanotic Xp11 translocation renal cancer is a more recently described entity. To date only 14 cases have been described. It is characterized by a distinct set of features including a nested epithelioid morphology, melanin pigmentation, labeling for markers of melanocytic differentiation, lack of labeling for markers of renal tubular differentiation, predominance in a younger age population and association with aggressive clinical behavior. There are noted similarities between that entity and TFE3 associated PEComas. There are no cases reported of equivalent melanotic TFEB translocation renal cancer. We report 2 rare cases of melanotic translocation renal neoplasms. The first is a melanotic TFE3 translocation renal cancer with an indolent clinical course, occurring in a patient more than 3-decades older than the usual average age in which such tumors have been described. The other case is, to our knowledge, the first reported melanotic TFEB translocation cancer of the kidney. Both cases exhibit the same H&E morphology as previously reported in melanotic translocation renal cancers and label accordingly with HMB45 and Melan-A. While the TFE3 melanotic tumor lacked any evidence of renal tubular differentiation, the TFEB melanotic cancer exhibited some staining for renal tubular markers. Based on the unique features noted above, these two cases expand the clinical and molecular spectrum of the melanotic translocation renal cancers. Copyright © 2017 Elsevier GmbH. All rights reserved.

  14. Novel deletion of lysine 7 expands the clinical, histopathological and genetic spectrum of TPM2-related myopathies

    Science.gov (United States)

    Davidson, Ann E.; Carlson, Heather A.; Moore, Brian E.; Love, Seth; Born, Donald E.; Roper, Helen; Majumdar, Anirban; Jayadev, Suman; Underhill, Hunter R.; Smith, Corrine O.; von der Hagen, Maja; Hubner, Angela; Jardine, Philip; Merrison, Andria; Curtis, Elizabeth; Cullup, Thomas; Jungbluth, Heinz; Cox, Mary O.; Winder, Thomas L.; Abdel Salam, Hossam; Li, Jun Z.; Moore, Steven A.; Dowling, James J.

    2013-01-01

    The β-tropomyosin gene encodes a component of the sarcomeric thin filament. Rod-shaped dimers of tropomyosin regulate actin-myosin interactions and β-tropomyosin mutations have been associated with nemaline myopathy, cap myopathy, Escobar syndrome and distal arthrogryposis types 1A and 2B. In this study, we expand the allelic spectrum of β-tropomyosin-related myopathies through the identification of a novel β-tropomyosin mutation in two clinical contexts not previously associated with β-tropomyosin. The first clinical phenotype is core-rod myopathy, with a β-tropomyosin mutation uncovered by whole exome sequencing in a family with autosomal dominant distal myopathy and muscle biopsy features of both minicores and nemaline rods. The second phenotype, observed in four unrelated families, is autosomal dominant trismus-pseudocamptodactyly syndrome (distal arthrogryposis type 7; previously associated exclusively with myosin heavy chain 8 mutations). In all four families, the mutation identified was a novel 3-bp in-frame deletion (c.20_22del) that results in deletion of a conserved lysine at the seventh amino acid position (p.K7del). This is the first mutation identified in the extreme N-terminus of β-tropomyosin. To understand the potential pathogenic mechanism(s) underlying this mutation, we performed both computational analysis and in vivo modelling. Our theoretical model predicts that the mutation disrupts the N-terminus of the α-helices of dimeric β-tropomyosin, a change predicted to alter protein–protein binding between β-tropomyosin and other molecules and to disturb head-to-tail polymerization of β-tropomyosin dimers. To create an in vivo model, we expressed wild-type or p.K7del β-tropomyosin in the developing zebrafish. p.K7del β-tropomyosin fails to localize properly within the thin filament compartment and its expression alters sarcomere length, suggesting that the mutation interferes with head-to-tail β-tropomyosin polymerization and with

  15. Dysautonomia, a heuristic approach to a revised model for etiology of disease.

    Science.gov (United States)

    Lonsdale, Derrick

    2009-03-01

    Dysautonomia refers to a disease where the autonomic nervous system is dysfunctional. This may be a central control mechanism, as in genetically determined familial dysautonomia (Riley-Day Syndrome), or peripherally in the distribution of the sympathetic and parasympathetic systems. There are multiple reports of a number of different diseases associated with dysautonomia. The etiology of this association has never been explained. There are also multiple publications on dysautonomia associated with specific non-caloric nutritional deficiencies. Beriberi is the prototype of autonomic dysfunction. It is the best known nutritional deficiency disease caused by an imbalance between ingested calories and the vitamins required for their oxidation, particularly thiamin. Long thought to be abolished in modern medical thinking, there are occasional isolated reports of the full-blown disease in developed Western cultures. Apart from genetically and epigenetically determined disease, evidence is presented that marginal high calorie malnutrition, particularly with reference to simple carbohydrates, is responsible for widespread dysautonomia. The brain and heart are the organs that have a fast rate of oxidative metabolism and are affected early by any mechanism that reduces oxidative efficiency. It is hypothesized that this results in a chaotic state of the hypothalamic/autonomic/endocrine axis. Due to the lack of adequate automatic controls, this may be responsible in some cases for breakdown of organ systems through long-standing energy deficiency, thus leading eventually to organic disease.

  16. Cold face test demonstrates parasympathetic cardiac dysfunction in familial dysautonomia.

    Science.gov (United States)

    Hilz, M J; Stemper, B; Sauer, P; Haertl, U; Singer, W; Axelrod, F B

    1999-06-01

    In familial dysautonomia (FD), i.e., Riley-Day syndrome, parasympathetic dysfunction has not been sufficiently evaluated. The cold face test is a noninvasive method of activating trigeminal brain stem cardiovagal and sympathetic pathways and can be performed in patients with limited cooperation. We performed cold face tests in 11 FD patients and 15 controls. For 60 s, cold compresses (0-1 degrees C) were applied to the cheeks and forehead while we monitored heart rate, respiration, beat-to-beat radial artery blood pressure, and laser-Doppler skin blood flow at the first toe pulp. From these measurements heart rate variability parameters were calculated: root mean square of successive differences (RMSSD), coefficient of variation (CV), low- and high-frequency (LF and HF, respectively) power spectra of the electrocardiogram, and the LF transfer function gain between blood pressure and heart rate. All patients perceived cold stimulation and acknowledged discomfort. In controls, heart rate and skin blood flow decreased significantly during cold face test; in patients, both parameters decreased only briefly and not significantly. In controls, blood pressure, RMSSD, CV, and heart rate HF-power spectra increased but remained unchanged in patients. Respiration, as well as heart rate LF power spectra, did not change in either group. In controls, LF transfer function gain between blood pressure and heart rate indicated that bradycardia was not secondary to blood pressure increase. We conclude that the cold face test demonstrated that patients with FD have a reduced cardiac parasympathetic response, which implies efferent parasympathetic dysfunction.

  17. Modelling familial dysautonomia in human induced pluripotent stem cells.

    Science.gov (United States)

    Lee, Gabsang; Studer, Lorenz

    2011-08-12

    Induced pluripotent stem (iPS) cells have considerable promise as a novel tool for modelling human disease and for drug discovery. While the generation of disease-specific iPS cells has become routine, realizing the potential of iPS cells in disease modelling poses challenges at multiple fronts. Such challenges include selecting a suitable disease target, directing the fate of iPS cells into symptom-relevant cell populations, identifying disease-related phenotypes and showing reversibility of such phenotypes using genetic or pharmacological approaches. Finally, the system needs to be scalable for use in modern drug discovery. Here, we will discuss these points in the context of modelling familial dysautonomia (FD, Riley-Day syndrome, hereditary sensory and autonomic neuropathy III (HSAN-III)), a rare genetic disorder in the peripheral nervous system. We have demonstrated three disease-specific phenotypes in FD-iPS-derived cells that can be partially rescued by treating cells with the plant hormone kinetin. Here, we will discuss how to use FD-iPS cells further in high throughput drug discovery assays, in modelling disease severity and in performing mechanistic studies aimed at understanding disease pathogenesis. FD is a rare disease but represents an important testing ground for exploring the potential of iPS cell technology in modelling and treating human disease.

  18. Spectrum

    DEFF Research Database (Denmark)

    Høgfeldt Hansen, Leif

    2016-01-01

    The publication functions as a proces description of the development and construction of an urban furniture SPECTRUM in the city of Gwangju, Republic of Korea. It is used as the cataloque for the exhibition of Spectrum.......The publication functions as a proces description of the development and construction of an urban furniture SPECTRUM in the city of Gwangju, Republic of Korea. It is used as the cataloque for the exhibition of Spectrum....

  19. Sympathetic and parasympathetic pupillary dysfunction in familial dysautonomia.

    Science.gov (United States)

    Dütsch, M; Hilz, M J; Rauhut, U; Solomon, J; Neundörfer, B; Axelrod, F B

    2002-03-15

    Objective assessment of autonomic dysfunction in familial dysautonomia (FD) is largely based on the analysis of cardiovascular responses to challenge maneuvers such as orthostatic stress. Infrared pupillometry (IPM) provides an additional reliable method for cranial autonomic evaluation and has the advantage of requiring minimal cooperation.This study was performed to determine whether IPM contributes to the assessment of autonomic function in FD patients. In 14 FD patients and 14 healthy controls, we studied absolute and relative light reflex amplitude, pupillary constriction velocity (v(constr)), pupillary diameter, early and late pupillary re-dilatation velocity (v(dil 1), v(dil 2)) after dark adaptation. Prior to IPM, all patients had an ophthamological examination to evaluate refraction and corneal integrity. In comparison to controls, patients had a significant reduction of the parameters reflecting parasympathetic pupillary function (absolute light reflex amplitude 1.34 +/- 0.21 vs. l.86 +/- 0.14 mm, relative light reflex amplitude 22.74 +/- 7.11% vs. 30.76 +/- 3.57%, v(constr) 3.75 +/- 1.09 vs. 5.80 +/- 0.59 mm/s) and of the parameters reflecting sympathetic pupillary function (diameter 5.69 +/- 0.66 vs. 6.35 +/- 0.60 mm, v(dil 1) 1.29 +/- 0.23 vs. 1.95 +/- 0.23 mm/s, v(dil 2) 0.64 +/- 0.13 vs. 0.72 +/- 0.l2 mm/s; Mann-Whitney U-test: p<0.05). The non-invasive technique of IPM demonstrates dysfunction not only of the cranial parasympathetic, but also of the cranial sympathetic nervous system and, thus, further characterizes autonomic dysfunction in FD.

  20. Alterations in amino acid status in cats with feline dysautonomia.

    Science.gov (United States)

    McGorum, Bruce C; Symonds, Herb W; Knottenbelt, Clare; Cave, Tom A; MacDonald, Susan J; Stratton, Joanna; Leon, Irene; Turner, Judith A; Pirie, R Scott

    2017-01-01

    Feline dysautonomia (FD) is a multiple system neuropathy of unknown aetiology. An apparently identical disease occurs in horses (equine grass sickness, EGS), dogs, rabbits, hares, sheep, alpacas and llamas. Horses with acute EGS have a marked reduction in plasma concentrations of the sulphur amino acids (SAA) cyst(e)ine and methionine, which may reflect exposure to a neurotoxic xenobiotic. The aim of this study was to determine whether FD cats have alterations in amino acid profiles similar to those of EGS horses. Amino acids were quantified in plasma/serum from 14 FD cats, 5 healthy in-contact cats which shared housing and diet with the FD cats, and 6 healthy control cats which were housed separately from FD cats and which received a different diet. The adequacy of amino acids in the cats' diet was assessed by determining the amino acid content of tinned and dry pelleted foods collected immediately after occurrences of FD. Compared with controls, FD cats had increased concentrations of many essential amino acids, with the exception of methionine which was significantly reduced, and reductions in most non-essential amino acids. In-contact cats also had inadequate methionine status. Artefactual loss of cysteine during analysis precluded assessment of the cyst(e)ine status. Food analysis indicated that the low methionine status was unlikely to be attributable to dietary inadequacy of methionine or cystine. Multi-mycotoxin screening identified low concentrations of several mycotoxins in dry food from all 3 premises. While this indicates fungal contamination of the food, none of these mycotoxins appears to induce the specific clinico-pathologic features which characterise FD and equivalent multiple system neuropathies in other species. Instead, we hypothesise that ingestion of another, as yet unidentified, dietary neurotoxic mycotoxin or xenobiotic, may cause both the characteristic disease pathology and the plasma SAA depletion.

  1. Alterations in amino acid status in cats with feline dysautonomia

    Science.gov (United States)

    Symonds, Herb W.; Knottenbelt, Clare; Cave, Tom A.; MacDonald, Susan J.; Stratton, Joanna; Leon, Irene; Turner, Judith A.; Pirie, R. Scott

    2017-01-01

    Feline dysautonomia (FD) is a multiple system neuropathy of unknown aetiology. An apparently identical disease occurs in horses (equine grass sickness, EGS), dogs, rabbits, hares, sheep, alpacas and llamas. Horses with acute EGS have a marked reduction in plasma concentrations of the sulphur amino acids (SAA) cyst(e)ine and methionine, which may reflect exposure to a neurotoxic xenobiotic. The aim of this study was to determine whether FD cats have alterations in amino acid profiles similar to those of EGS horses. Amino acids were quantified in plasma/serum from 14 FD cats, 5 healthy in-contact cats which shared housing and diet with the FD cats, and 6 healthy control cats which were housed separately from FD cats and which received a different diet. The adequacy of amino acids in the cats’ diet was assessed by determining the amino acid content of tinned and dry pelleted foods collected immediately after occurrences of FD. Compared with controls, FD cats had increased concentrations of many essential amino acids, with the exception of methionine which was significantly reduced, and reductions in most non-essential amino acids. In-contact cats also had inadequate methionine status. Artefactual loss of cysteine during analysis precluded assessment of the cyst(e)ine status. Food analysis indicated that the low methionine status was unlikely to be attributable to dietary inadequacy of methionine or cystine. Multi-mycotoxin screening identified low concentrations of several mycotoxins in dry food from all 3 premises. While this indicates fungal contamination of the food, none of these mycotoxins appears to induce the specific clinico-pathologic features which characterise FD and equivalent multiple system neuropathies in other species. Instead, we hypothesise that ingestion of another, as yet unidentified, dietary neurotoxic mycotoxin or xenobiotic, may cause both the characteristic disease pathology and the plasma SAA depletion. PMID:28333983

  2. 24-hour ambulatory blood pressure monitoring in children with familial dysautonomia.

    Science.gov (United States)

    Nussinovitch, Naomi; Nussinovitch, Moshe; Peleg, Edna; Rosenthal, Talma

    2005-04-01

    Familial dysautonomia (Riley Day syndrome) is a genetic disease. The present study of 24-hour ambulatory blood pressure monitoring in children with familial dysautonomia was carried out to investigate the pattern of blood pressure in this syndrome. To the best of our knowledge, this is the only description of patients with 24-hour blood pressure monitoring. Vasomotor instability reflected in extreme hypertension and hypotension was recorded by 24-hour ambulatory blood pressure monitoring in three patients with familial dysautonomia: a 16-year old girl, a 14-year old boy and a 3-year old boy. Recordings were taken on a routine school day in the first two patients and during hospitalization in the third. Patients 1 and 2 displayed circadian rhythm but with significantly higher than normal blood pressure and heart rate. Patient 3 exhibited these fluctuations to a lesser degree. Postural hypotension without compensatory tachycardia was frequently seen in all three patients. Unusual variability in blood pressure was recorded during routine activities in patients 1 and 2 and during an acute attack in patient 3. Close monitoring of antihypertensive therapy should be considered in familial dysautonomia patients in whom blood pressure reaches excessive levels.

  3. Cardiac-locked bursts of muscle sympathetic nerve activity are absent in familial dysautonomia.

    Science.gov (United States)

    Macefield, Vaughan G; Norcliffe-Kaufmann, Lucy; Axelrod, Felicia B; Kaufmann, Horacio

    2013-02-01

    Familial dysautonomia (Riley-Day syndrome) is an hereditary sensory and autonomic neuropathy (HSAN type III), expressed at birth, that is associated with reduced pain and temperature sensibilities and absent baroreflexes, causing orthostatic hypotension as well as labile blood pressure that increases markedly during emotional excitement. Given the apparent absence of functional baroreceptor afferents, we tested the hypothesis that the normal cardiac-locked bursts of muscle sympathetic nerve activity (MSNA) are absent in patients with familial dysautonomia. Tungsten microelectrodes were inserted percutaneously into muscle or cutaneous fascicles of the common peroneal nerve in 12 patients with familial dysautonomia. Spontaneous bursts of MSNA were absent in all patients, but in five patients we found evidence of tonically firing sympathetic neurones, with no cardiac rhythmicity, that increased their spontaneous discharge during emotional arousal but not during a manoeuvre that unloads the baroreceptors. Conversely, skin sympathetic nerve activity (SSNA), recorded in four patients, appeared normal. We conclude that the loss of phasic bursts of MSNA and the loss of baroreflex modulation of muscle vasoconstrictor drive contributes to the poor control of blood pressure in familial dysautonomia, and that the increase in tonic firing of muscle vasoconstrictor neurones contributes to the increase in blood pressure during emotional excitement.

  4. Nutritional risk and gastrointestinal dysautonomia symptoms in Parkinson's disease outpatients hospitalised on a scheduled basis.

    Science.gov (United States)

    Barichella, Michela; Cereda, Emanuele; Madio, Carmen; Iorio, Laura; Pusani, Chiara; Cancello, Raffaella; Caccialanza, Riccardo; Pezzoli, Gianni; Cassani, Erica

    2013-07-28

    Dysautonomia symptoms of nutritional interest may often occur in Parkinson's disease (PD), but the role played in affecting the risk of malnutrition still needs to be clarified. A total of 208 consecutive PD outpatients hospitalised on a scheduled basis were assessed for nutritional risk by the Malnutrition Universal Screening Tool. Presence of dysautonomia symptoms (dysphagia, sialorrhoea and constipation) was investigated using clinical rating scales. In our population, prevalence of nutritional risk was 17·2 (95 % CI 12·1, 24·0) % and relied mainly on unintentional weight loss. Sialorrhoea, dysphagia, dysphagia to liquids and constipation were observed in 10·6, 11·0, 14·4 and 59·6 % of the patients, respectively. Nutritional risk was independently associated with the number of dysautonomia symptoms (OR 1·39 (95 % CI 1·00, 1·96); P= 0·048) but not with single symptoms. An independent association was also found with the severity of motor symptoms (Hoehn-Yahr stage, OR 1·48 (95 % CI 1·00, 2·55); P= 0·049) and levodopa dose (OR 1·16 (95 % CI 1·04, 1·31) mg/kg per d; P= 0·009). Nutritional risk in PD outpatients appears to depend mainly on dysautonomic syndrome, disease severity and levodopa dosage. Implications for outcome deserve further investigation. The assessment of nutritional status and of gastrointestinal dysautonomia symptoms should be part of the routine work-up of a PD patient.

  5. Expanding the clinical spectrum of the 16p11.2 chromosomal rearrangements: three patients with syringomyelia.

    Science.gov (United States)

    Schaaf, Christian P; Goin-Kochel, Robin P; Nowell, Kerri P; Hunter, Jill V; Aleck, Kirk A; Cox, Sarah; Patel, Ankita; Bacino, Carlos A; Shinawi, Marwan

    2011-02-01

    16p11.2 rearrangements are associated with developmental delay, cognitive impairment, autism spectrum disorder, behavioral problems (especially attention-deficit hyperactivity disorder), seizures, obesity, dysmorphic features, and abnormal head size. In addition, congenital anomalies and abnormal brain findings were frequently observed in patients with these rearrangements. We identified and performed a detailed microarray, phenotypic, and radiological characterization of three new patients with 16p11.2 rearrangements: two deletion patients and one patient with the reciprocal duplication. All patients have a heterozygous loss (deletion) or gain (duplication) corresponding to chromosomal coordinates (chr16: 29 528 190-30 107 184) with a minimal size of 579 kb. The deletion patients had language delay and learning disabilities and one met criteria for pervasive developmental disorder not otherwise specified. The duplication patient received a diagnosis of autism and had academic deficits and behavioral problems. The patients with deletion had long cervicothoracic syringomyelia and the duplication patient had long thoracolumbar syringomyelia. The syringomyelia in one patient with deletion was associated with Chiari malformation. Our findings highlight the broad spectrum of clinical and neurological manifestations in patients with 16p11.2 rearrangements. Our observation suggests that genes (or a single gene) within the implicated interval have significant roles in the pathogenesis of syringomyelia. A more comprehensive and systematic research is warranted to study the frequency and spectrum of malformations in the central nervous system in these patients.

  6. Tocotrienol Treatment in Familial Dysautonomia: Open-Label Pilot Study.

    Science.gov (United States)

    Cheishvili, David; Maayan, Channa; Holzer, Naama; Tsenter, Jeanna; Lax, Elad; Petropoulos, Sophie; Razin, Aharon

    2016-07-01

    Familial dysautonomia (FD) is an autosomal recessive congenital neuropathy, primarily presented in Ashkenazi Jews. The most common mutation in FD patients results from a single base pair substitution of an intronic splice site in the IKBKAP gene which disrupts normal mRNA splicing and leads to tissue-specific reduction of IKBKAP protein (IKAP). To date, treatment of FD patients remains preventative, symptomatic and supportive. Based on previous in vitro evidence that tocotrienols, members of the vitamin E family, upregulate transcription of the IKBKAP gene, we aimed to investigate whether a similar effects was observed in vivo. In the current study, we assessed the effects of tocotrienol treatment on FD patients' symptoms and IKBKAP expression in white blood cells. The initial daily doses of 50 or 100 mg tocotrienol, doubled after 3 months, was administered to 32 FD patients. Twenty-eight FD patients completed the 6-month study. The first 3 months of tocotrienol treatment was associated with a significant increase in IKBKAP expression level in FD patients' blood. Despite doubling the dose after the initial 3 months of treatment, IKBKAP expression level returned to baseline by the end of the 6-month treatment. Clinical improvement was noted in the reported clinical questionnaire (with regard to dizziness, bloching, sweating, number of pneumonia, cough episodes, and walking stability), however, no significant effect was observed in any clinical measurements (weight, height, oxygen saturation, blood pressure, tear production, histamine test, vibration threshold test, nerve conduction, and heart rate variability) following Tocotrienol treatment. In conclusion, tocotrienol treatment appears significantly beneficial by clinical evaluation for some FD patients in a few clinical parameters; however it was not significant by clinical measurements. This open-label study shows the complexity of effect of tocotrienol treatment on FD patients' clinical outcomes and on

  7. Rhabdoid Variant of Myoepithelial Carcinoma, with EWSR1 Rearrangement: Expanding the Spectrum of EWSR1-Rearranged Myoepithelial Tumors.

    Science.gov (United States)

    Thway, Khin; Bown, Nick; Miah, Aisha; Turner, Rob; Fisher, Cyril

    2015-06-01

    Myoepithelial and mixed tumors represent a heterogeneous group of neoplasms for which classification is incomplete and continues to evolve. Those arising in the soft tissues appear to represent subgroups that are genetically distinct from those that occur within salivary glands. We describe a case of soft tissue myoepithelial carcinoma with rhabdoid morphology, which presented as an enlarging neck mass in a 40 year old male, and in which EWSR1 rearrangement was demonstrated by fluorescence in situ hybridization. This neoplasm showed diffuse INI1 loss, making distinction from other INI1-negative rhabdoid tumors difficult. This expands the range of reported histologic features of EWSR1-rearranged myoepithelial neoplasms, and highlights the significant morphologic and immunohistochemical overlap between this and other INI1-negative malignant rhabdoid neoplasms.

  8. A de novo dominant mutation in ACTA1 causing congenital nemaline myopathy associated with a milder phenotype: expanding the spectrum of dominant ACTA1 mutations.

    Science.gov (United States)

    Levesque, L; Del Bigio, M R; Krawitz, S; Mhanni, A A

    2013-03-01

    We describe the presentation and six-year follow up of a child with nemaline myopathy due to a de novo mutation in the skeletal muscle α-actin gene (ACTA1) characterized by dramatic improvement during the early childhood years. The presentation in this female patient was infantile-onset weakness in the facial, bulbar, respiratory and neck flexor muscles. A six-year follow-up revealed continued progressive improvement in her muscle strength. Based upon the histopathologic and ultrastructural features of nemaline rod disease, ACTA1 was sequenced. This revealed a mutation in exon 4 of ACTA1 (c.557A>G). Our report further expands the phenotypic spectrum associated with ACTA1 mutations. Although it is difficult to infer any genotype-phenotype correlation, this report stimulates the discussion regarding the pathophysiologic mechanism of the clinical improvement seen in some patients with ACTA1 mutations. Copyright © 2012 Elsevier B.V. All rights reserved.

  9. Post-adenoviral parasympathetic dysautonomia in a child: a case report

    Directory of Open Access Journals (Sweden)

    Martinez Adalaida

    2010-06-01

    Full Text Available Abstract Introduction This is the first reported case of adenoviral meningoencephalitis that was complicated by persistent parasympathetic dysautonomia, which clinically either stimulated or inhibited its activity. Case presentation A 7-year-old Caucasian girl presented to our hospital in March 2008 with a three day history of upper respiratory infection. Her condition worsened and she was placed on ventilator support for two weeks. Her recovery was complicated by a persistent selective parasympathetic dysautonomia. Her past medical history was unremarkable. Conclusions To the best of our knowledge this is the first case of an adenoviral infection in a child which was complicated after recovery from an acute meningoencephalitis and peripheral neuropathy.

  10. 6-[18F]Fluoro-L-DOPA: A Well-Established Neurotracer with Expanding Application Spectrum and Strongly Improved Radiosyntheses

    Directory of Open Access Journals (Sweden)

    M. Pretze

    2014-01-01

    Full Text Available For many years, the main application of [18F]F-DOPA has been the PET imaging of neuropsychiatric diseases, movement disorders, and brain malignancies. Recent findings however point to very favorable results of this tracer for the imaging of other malignant diseases such as neuroendocrine tumors, pheochromocytoma, and pancreatic adenocarcinoma expanding its application spectrum. With the application of this tracer in neuroendocrine tumor imaging, improved radiosyntheses have been developed. Among these, the no-carrier-added nucleophilic introduction of fluorine-18, especially, has gained increasing attention as it gives [18F]F-DOPA in higher specific activities and shorter reaction times by less intricate synthesis protocols. The nucleophilic syntheses which were developed recently are able to provide [18F]F-DOPA by automated syntheses in very high specific activities, radiochemical yields, and enantiomeric purities. This review summarizes the developments in the field of [18F]F-DOPA syntheses using electrophilic synthesis pathways as well as recent developments of nucleophilic syntheses of [18F]F-DOPA and compares the different synthesis strategies regarding the accessibility and applicability of the products for human in vivo PET tumor imaging.

  11. De novo mutations in beta-catenin (CTNNB1) appear to be a frequent cause of intellectual disability: expanding the mutational and clinical spectrum.

    Science.gov (United States)

    Kuechler, Alma; Willemsen, Marjolein H; Albrecht, Beate; Bacino, Carlos A; Bartholomew, Dennis W; van Bokhoven, Hans; van den Boogaard, Marie Jose H; Bramswig, Nuria; Büttner, Christian; Cremer, Kirsten; Czeschik, Johanna Christina; Engels, Hartmut; van Gassen, Koen; Graf, Elisabeth; van Haelst, Mieke; He, Weimin; Hogue, Jacob S; Kempers, Marlies; Koolen, David; Monroe, Glen; de Munnik, Sonja; Pastore, Matthew; Reis, André; Reuter, Miriam S; Tegay, David H; Veltman, Joris; Visser, Gepke; van Hasselt, Peter; Smeets, Eric E J; Vissers, Lisenka; Wieland, Thomas; Wissink, Willemijn; Yntema, Helger; Zink, Alexander Michael; Strom, Tim M; Lüdecke, Hermann-Josef; Kleefstra, Tjitske; Wieczorek, Dagmar

    2015-01-01

    Recently, de novo heterozygous loss-of-function mutations in beta-catenin (CTNNB1) were described for the first time in four individuals with intellectual disability (ID), microcephaly, limited speech and (progressive) spasticity, and functional consequences of CTNNB1 deficiency were characterized in a mouse model. Beta-catenin is a key downstream component of the canonical Wnt signaling pathway. Somatic gain-of-function mutations have already been found in various tumor types, whereas germline loss-of-function mutations in animal models have been shown to influence neuronal development and maturation. We report on 16 additional individuals from 15 families in whom we newly identified de novo loss-of-function CTNNB1 mutations (six nonsense, five frameshift, one missense, two splice mutation, and one whole gene deletion). All patients have ID, motor delay and speech impairment (both mostly severe) and abnormal muscle tone (truncal hypotonia and distal hypertonia/spasticity). The craniofacial phenotype comprised microcephaly (typically -2 to -4 SD) in 12 of 16 and some overlapping facial features in all individuals (broad nasal tip, small alae nasi, long and/or flat philtrum, thin upper lip vermillion). With this detailed phenotypic characterization of 16 additional individuals, we expand and further establish the clinical and mutational spectrum of inactivating CTNNB1 mutations and thereby clinically delineate this new CTNNB1 haploinsufficiency syndrome.

  12. Tissue-Specific Expression of a Splicing Mutation in the IKBKAP Gene Causes Familial Dysautonomia

    OpenAIRE

    Slaugenhaupt, Susan A; Blumenfeld, Anat; Gill, Sandra P.; Leyne, Maire; Mull, James; Cuajungco, Math P.; Liebert, Christopher B.; Chadwick, Brian; Idelson, Maria; Reznik, Luba; Robbins, Christiane M.; Makalowska, Izabela; Brownstein, Michael J.; Krappmann, Daniel; Scheidereit, Claus

    2001-01-01

    Familial dysautonomia (FD; also known as “Riley-Day syndrome”), an Ashkenazi Jewish disorder, is the best known and most frequent of a group of congenital sensory neuropathies and is characterized by widespread sensory and variable autonomic dysfunction. Previously, we had mapped the FD gene, DYS, to a 0.5-cM region on chromosome 9q31 and had shown that the ethnic bias is due to a founder effect, with >99.5% of disease alleles sharing a common ancestral haplotype. To investigate the molecular...

  13. Abundance and phenotypic diversity of Escherichia coli isolates with diminished susceptibility to expanded-spectrum cephalosporins in faeces from healthy food animals after slaughter.

    Science.gov (United States)

    Moreno, Miguel A; Teshager, Tirushet; Porrero, M A Concepción; García, María; Escudero, Esther; Torres, Carmen; Domínguez, Lucas

    2007-03-10

    Antimicrobial resistance (AR) is an increasing phenomenon but its quantitative estimation remains controversial. The classical resistance percentage approach is not well suited to detect either emergence or low levels resistance. One option is to shift the focus from strains to hosts. This approach is applied to test for phenotypic diversity associated with diminished susceptibility to expanded-spectrum cephalosporins (DSESC) in faecal Escherichia coli from healthy food animals in Spain. We performed E. coli enumeration in faecal samples of broilers (82 pooled samples) and pigs (80 pooled samples) at the slaughterhouse level, using Coli-ID plates alone and supplemented with cefotaxime at two levels (1 and 8 microg/ml). Antimicrobial susceptibility of isolates was tested by the agar diffusion method. Clustering was carried out using these numerical values and Ward and UPGMA methods. When using plates supplemented with 1 microg/ml of cefotaxime for DSESC E. coli detection, 93% (76/82) of broiler pooled samples and 36% (29/80) pig pooled samples tested positive. When using 8 microg/ml of cefotaxime, 67% (55/82) of broilers and 13% (10/80) of pigs were positive. Nevertheless, the relative abundance of this phenotype was low in both animal species (range 0-4.3%). Irrespective of the clustering method (Ward or UPGMA), a noticeable phenotypic diversity was detected, especially from the plates containing 1 microg/ml of cefotaxime. We concluded that: (a) E. coli with phenotype DSESC are common in broilers and pigs but are less frequent in pigs, and (b) the host approach is the most appropriate method for antimicrobial resistance assessment when null or very low levels of antimicrobial resistant bacteria are expected.

  14. Expanding the mutation spectrum in 130 probands with ARPKD: identification of 62 novel PKHD1 mutations by sanger sequencing and MLPA analysis.

    Science.gov (United States)

    Melchionda, Salvatore; Palladino, Teresa; Castellana, Stefano; Giordano, Mario; Benetti, Elisa; De Bonis, Patrizia; Zelante, Leopoldo; Bisceglia, Luigi

    2016-09-01

    Autosomal recessive polycystic kidney disease (ARPKD) is a rare severe genetic disorder arising in the perinatal period, although a late-onset presentation of the disease has been described. Pulmonary hypoplasia is the major cause of morbidity and mortality in the newborn period. ARPKD is caused by mutations in the PKHD1 (polycystic kidney and hepatic disease 1) gene that is among the largest human genes. To achieve a molecular diagnosis of the disease, a large series of Italian affected subjects were recruited. Exhaustive mutation analysis of PKHD1 gene was carried out by Sanger sequencing and multiple ligation probe amplification (MLPA) technique in 110 individuals. A total of 173 mutations resulting in a detection rate of 78.6% were identified. Additional 20 unrelated patients, in whom it was not possible to analyze the whole coding sequence, have been included in this study. Taking into account the total number (n=130) of this cohort of patients, 107 different types of mutations have been detected in 193 mutated alleles. Out of 107 mutations, 62 were novel: 11 nonsense, 6 frameshift, 7 splice site mutations, 2 in-frame deletions and 2 multiexon deletion detected by MLPA. Thirty-four were missense variants. In conclusion, our report expands the spectrum of PKHD1 mutations and confirms the heterogeneity of this disorder. The population under study represents the largest Italian ARPKD cohort reported to date. The estimated costs and the time invested for molecular screening of genes with large size and allelic heterogeneity such as PKHD1 demand the use of next-generation sequencing (NGS) technologies for a faster and cheaper screening of the affected subjects.

  15. Familial dysautonomia (Riley-Day syndrome). Temporal bone findings and otolaryngological manifestations.

    Science.gov (United States)

    Tokita, N; Sekhar, H K; Sachs, M; Daly, J F

    1978-01-01

    Familial dysautonomia, or Riley-Day syndrome, is inherited in an autosomal recessive fashion and occurs almost exclusively in Jewish families. This disorder is characterized by a smooth tongue devoid of fungiform papillae and of taste buds, and is clinically associated with poor taste discrimination. An unsteady gait and dizziness on change in position are also common presenting symptoms. This study reports the histopathological findings of eight temporal bones from four patients with documented familial dysautonomia. For the control series, 13 normal temporal bones were also studied. The most striking finding in the dysautonomic patients was an extreme paucity of geniculate ganglion cells (P less than 0.001). A statistically significant reduction in the number of neurons was also found both in the superior and in the inferior divisions of the vestibular nerve (P less than 0.001). The paucity of the geniculate ganglion cells correlates well with the impairment of the taste in dysautonomic individuals, since the afferent fibers leaving taste buds of the anterior two-thirds of the tongue run via the chorda tympani and have their cell bodies in the geniculate ganglion. Furthermore, the reduction in the number of Scarpa's ganglion cells observed in the dysautonomic patients studied here could account for a poor response to caloric test, positional vertigo and an unsteady gait in this condition.

  16. Structural abnormalities of the myenteric (Auerbach's) plexus in familial dysautonomia (Riley-Day syndrome) as demonstrated by flat-mount preparation of the esophagus and stomach.

    Science.gov (United States)

    Ariel, I; Wells, T R

    1985-01-01

    The esophageal and gastric myenteric (Auerbach's) plexus were studied by a flat-mount preparation in 3 patients with familial dysautonomia. In 1 patient a typical esophageal network was found in the stomach. In another patient both esophageal and gastric plexus patterns were significantly different from normal. These changes, by producing abnormal nervous stimulation, may explain disturbed motility of the esophagus and stomach in familial dysautonomia.

  17. Renal salt wasting as part of dysautonomia in Guillain-Barre syndrome.

    Science.gov (United States)

    Lenhard, T; Grimm, C; Ringleb, P A

    2011-09-01

    Cerebral salt-wasting syndrome and the syndrome of inappropriate antidiuresis (SIAD) are the most important causes of non-iatrogenic hyponatraemia that can significantly complicate various brain diseases. Salt wasting without an underlying CNS disease may have been disregarded so far by clinicians and has been described as renal salt-wasting (RSW) in patients as drug side effect (eg, cisplatin), in older people with various common diseases (eg, hip fracture, pulmonary infections) and other sporadic conditions. In Guillain-Barré Syndrome (GBS), however, hyponatraemia has been described mainly as SIAD. However, symptoms of hyponatraemia rarely develop in GBS. Here, we report on a woman with GBS with dominant symptoms of dysautonomia and moderate severe hyponatraemia. We could identify RSW as part of the autonomic dysfunction that significantly contributed to disease worsening.

  18. Trends in Expanded-Spectrum Cephalosporin-Resistant Escherichia coli and Klebsiella pneumoniae among Dutch Clinical Isolates, from 2008 to 2012

    NARCIS (Netherlands)

    van der Steen, Matthijs; Leenstra, Tjalling; Kluytmans, Jan A J W; van der Bij, Akke K

    2015-01-01

    We investigated time trends in extended-spectrum cephalosporin-resistant Escherichia coli and Klebsiella pneumoniae isolates from different patient settings in The Netherlands from 2008-2012. E. coli and K. pneumoniae isolates from blood and urine samples of patients > = 18 years were selected from

  19. Trends in Expanded-Spectrum Cephalosporin-Resistant Escherichia coli and Klebsiella pneumoniae among Dutch Clinical Isolates, from 2008 to 2012

    NARCIS (Netherlands)

    van der Steen, Matthijs; Leenstra, Tjalling; Kluytmans, Jan A J W; van der Bij, Akke K

    2015-01-01

    We investigated time trends in extended-spectrum cephalosporin-resistant Escherichia coli and Klebsiella pneumoniae isolates from different patient settings in The Netherlands from 2008-2012. E. coli and K. pneumoniae isolates from blood and urine samples of patients > = 18 years were selected from

  20. Expanding the phenotypic spectrum of ARID1B-mediated disorders and identification of altered cell-cycle dynamics due to ARID1B haploinsufficiency

    DEFF Research Database (Denmark)

    Sim, J. C. H.; White, S. M.; Fitzpatrick, E.

    2014-01-01

    or nonsense mutations in all of them. Conclusions: This study broadens the spectrum of ARID1B associated phenotypes by describing a distinctive phenotype including plantar fat pads but lacking the hypertrichosis or fifth nail hypoplasia associated with Coffin-Siris syndrome. We present the first direct...

  1. Trends in Expanded-Spectrum Cephalosporin-Resistant Escherichia coli and Klebsiella pneumoniae among Dutch Clinical Isolates, from 2008 to 2012

    Science.gov (United States)

    van der Steen, Matthijs; Leenstra, Tjalling; Kluytmans, Jan A. J. W.; van der Bij, Akke K.

    2015-01-01

    We investigated time trends in extended-spectrum cephalosporin-resistant Escherichia coli and Klebsiella pneumoniae isolates from different patient settings in The Netherlands from 2008–2012. E. coli and K. pneumoniae isolates from blood and urine samples of patients > = 18 years were selected from the Dutch Infectious Disease Surveillance System-Antimicrobial Resistance (ISIS-AR) database. We used multivariable Poisson regression to study the rate per year of blood stream infections by susceptible and resistant isolates, and generalized estimating equation (GEE) log-binomial regression for trends in the proportion of extended-spectrum cephalosporin-resistant isolates. Susceptibility data of 197,513 E. coli and 38,244 K. pneumoniae isolates were included. The proportion of extended-spectrum cephalosporin-resistant E. coli and K. pneumoniae isolates from urine and blood samples increased in all patient settings, except for K. pneumoniae isolates from patients admitted to intensive care units. For K. pneumoniae, there was a different time trend between various patient groups (p<0.01), with a significantly higher increase in extended-spectrum cephalosporin-resistant isolates from patients attending a general practitioner than in isolates from hospitalized patients. For E. coli, the increasing time trends did not differ among different patient groups. This nationwide study shows a general increase in extended-spectrum cephalosporin-resistant E. coli and K. pneumoniae isolates. However, differences in trends between E. coli en K. pneumoniae underline the importance of E. coli as a community-pathogen and its subsequent influence on hospital resistance level, while for K. pneumoniae the level of resistance within the hospital seems less influenced by the resistance trends in the community. PMID:26381746

  2. Aspects of spinal deformity in familial dysautonomia (Riley-Day syndrome).

    Science.gov (United States)

    Kaplan, L; Margulies, J Y; Kadari, A; Floman, Y; Robin, G C

    1997-01-01

    Familial dysautonomia (FD) is a rare autosomal recessive disease occurring in Jews of Ashkenazi descent, with only some 500 recognized cases. The causative gene was identified on chromosome 9. FD is of considerable orthopedic interest, because of the prevalence of skeletal deformity. About 90% of surviving dysautonomic children will develop a spinal curvature, commonly a scoliosis. The scoliotic curve is usually kyphotic rather than lordotic, and appears during the first decade of life. Fifty-one of the 90 reported cases of familial dysautonomia in Israel involved patients who were seen at the scoliosis clinic for assessment and treatment of their spinal deformities. Most of the patients presented with a scoliotic deformity associated in 37 cases with an increased thoracic kyphosis. In our series orthotic treatment and physiotherapy were found to be minimally successful at best. Surgical treatment of the spine was performed in 13 of 51 patients in this series. A retrospective review of these patients' charts and radiographs was carried out. Six years of follow-up are reported. The primary indication for surgery was progression of the spinal curve. Only posterior spinal fusions were performed. Anterior transthoracic procedures were avoided in spite of the significance of the kyphotic deformity, because of the frequency of pulmonary complications. Harrington distraction and compression instrumentation was used. Three-millimeter compression rods were used in a distraction mode in thin, young children. "Harri-Luque" segmental sublaminar wiring technique and Wisconsin spinous process segmental wiring was used in some. In all cases, the spine fusion was supplemented by bank bone only, to avoid the additional trauma of graft removal. We believe that surgical intervention is advantageous, if done early in the evolution of spinal deformity. Greater technical difficulties and a higher complication rate were encountered in this series relative to the problems usually seen

  3. Expanding earth

    Energy Technology Data Exchange (ETDEWEB)

    Carey, S.W.

    1976-01-01

    Arguments in favor of an expanding earth are presented. The author believes that the theory of plate tectonics is a classic error in the history of geology. The case for the expanding earth is organized in the following way: introductory review - face of the earth, development of expanding earth concept, necessity for expansion, the subduction myth, and definitions; some principles - scale of tectonic phenomena, non-uniformitarianism, tectonic profile, paleomagnetism, asymmetry of the earth, rotation of the earth, and modes of crustal extension; regional studies - western North America, Central America, South-East Asia, and the rift oceans; tests and cause of expansion. 824 references, 197 figures, 11 tables. (RWR)

  4. Kinetin in familial dysautonomia carriers: implications for a new therapeutic strategy targeting mRNA splicing.

    Science.gov (United States)

    Gold-von Simson, Gabrielle; Goldberg, Judith D; Rolnitzky, Linda M; Mull, James; Leyne, Maire; Voustianiouk, Andrei; Slaugenhaupt, Susan A; Axelrod, Felicia B

    2009-03-01

    Familial dysautonomia (FD) is caused by an intronic splice mutation in the IkappaB kinase-associated protein gene (IKBKAP) that leads to partial skipping of exon 20 and tissue-specific reduction of IkappaB kinase-associated protein/elongator protein 1 (IKAP/ELP-1 protein). Kinetin increases IKBKAP mRNA and protein expression in FD cell lines. To determine whether oral kinetin alters IKBKAP splicing in vivo, we administered kinetin to 29 healthy carriers of the major FD mutation for 8 d. Adverse effects, kinetin, and IKBKAP mRNA levels were monitored. In the highest dosing cohorts (23.5 mg/kg/d), the target plasma kinetin level was achieved in 91% of subjects at 2 h. After 8 d, IKBKAP mRNA expression in leukocytes increased as kinetin levels increased. There is a linear association between log plasma kinetin level and corresponding log change from baseline in IKBKAP mRNA expression that allows estimation of IKBKAP mRNA levels because of kinetin ingestion. Adverse effects were transient and mild. This is the first report of in vivo IKBKAP splicing modification and strongly suggests kinetin's therapeutic potential in FD and perhaps in other splicing disorders. Furthermore, our findings support our hypothesis that treatments, which target a particular splicing mutation, can be successfully developed.

  5. Kinetin improves IKBKAP mRNA splicing in patients with familial dysautonomia.

    Science.gov (United States)

    Axelrod, Felicia B; Liebes, Leonard; Gold-Von Simson, Gabrielle; Mendoza, Sandra; Mull, James; Leyne, Maire; Norcliffe-Kaufmann, Lucy; Kaufmann, Horacio; Slaugenhaupt, Susan A

    2011-11-01

    Familial dysautonomia (FD) is caused by an intronic splice mutation in the IKBKAP gene that leads to partial skipping of exon 20 and tissue-specific reduction in I-κ-B kinase complex-associated protein/elongation protein 1 (IKAP/ELP-1) expression. Kinetin (6-furfurylaminopurine) has been shown to improve splicing and increase WT IKBKAP mRNA and IKAP protein expression in FD cell lines and carriers. To determine whether oral kinetin treatment could alter mRNA splicing in FD subjects and was tolerable, we administered kinetin to eight FD individuals homozygous for the splice mutation. Subjects received 23.5 mg/Kg/d for 28 d. An increase in WT IKBKAP mRNA expression in leukocytes was noted after 8 d in six of eight individuals; after 28 d, the mean increase compared with baseline was significant (p = 0.002). We have demonstrated that kinetin is tolerable in this medically fragile population. Not only did kinetin produce the desired effect on splicing in FD patients but also that effect seems to improve with time despite lack of dose change. This is the first report of a drug that produces in vivo mRNA splicing changes in individuals with FD and supports future long-term trials to determine whether kinetin will prove therapeutic in FD patients.

  6. Tissue-specific expression of a splicing mutation in the IKBKAP gene causes familial dysautonomia.

    Science.gov (United States)

    Slaugenhaupt, S A; Blumenfeld, A; Gill, S P; Leyne, M; Mull, J; Cuajungco, M P; Liebert, C B; Chadwick, B; Idelson, M; Reznik, L; Robbins, C; Makalowska, I; Brownstein, M; Krappmann, D; Scheidereit, C; Maayan, C; Axelrod, F B; Gusella, J F

    2001-03-01

    Familial dysautonomia (FD; also known as "Riley-Day syndrome"), an Ashkenazi Jewish disorder, is the best known and most frequent of a group of congenital sensory neuropathies and is characterized by widespread sensory and variable autonomic dysfunction. Previously, we had mapped the FD gene, DYS, to a 0.5-cM region on chromosome 9q31 and had shown that the ethnic bias is due to a founder effect, with >99.5% of disease alleles sharing a common ancestral haplotype. To investigate the molecular basis of FD, we sequenced the minimal candidate region and cloned and characterized its five genes. One of these, IKBKAP, harbors two mutations that can cause FD. The major haplotype mutation is located in the donor splice site of intron 20. This mutation can result in skipping of exon 20 in the mRNA of patients with FD, although they continue to express varying levels of wild-type message in a tissue-specific manner. RNA isolated from lymphoblasts of patients is primarily wild-type, whereas only the deleted message is seen in RNA isolated from brain. The mutation associated with the minor haplotype in four patients is a missense (R696P) mutation in exon 19, which is predicted to disrupt a potential phosphorylation site. Our findings indicate that almost all cases of FD are caused by an unusual splice defect that displays tissue-specific expression; and they also provide the basis for rapid carrier screening in the Ashkenazi Jewish population.

  7. Properties of herbage in relation to equine dysautonomia: biochemical composition and antioxidant and prooxidant actions.

    Science.gov (United States)

    McGorum, B C; Fry, S C; Wallace, G; Coenen, K; Robb, J; Williamson, G; Aruoma, O I

    2000-06-01

    To investigate the etiology of equine dysautonomia (ED), a degenerative polyneuropathy affecting grazing horses, the biochemical composition and antioxidant/prooxidant activities of aqueous extracts of plants collected from ED pastures were determined. Plants collected immediately after an outbreak of ED had reduced antioxidant and weak prooxidant activities when compared with control plants (plants collected from ED pastures out of ED season and control plants from ED pastures that were grown under favorable conditions). ED plants also had significantly increased concentrations of fructose and low molecular weight phenolic compounds, significantly more of one amino acid zone (probably valine), significantly less tartaric acid, and a nonsignificant decrease in ascorbic acid content when compared with control plants from ED pastures that were grown under favorable conditions. These findings suggest that ED plants may be under oxidative stress, possibly due to chilling, drought, or fungal colonization. However, experimental drought and chilling of plants did not reproduce the biochemical alterations identified in ED plants. It is possible that the altered biochemical content of ingested plants may contribute, directly or indirectly, to the development of ED in grazing horses.

  8. Familial dysautonomia's impact on quality of life in childhood, adolescence, and adulthood.

    Science.gov (United States)

    Sands, Stephen A; Giarraffa, Philip; Jacobson, Colleen M; Axelrod, Felicia B

    2006-04-01

    To evaluate the quality of life (QoL) of children, adolescents, and adults treated for familial dysautonomia (FD), a pervasive neurological disorder. The Child Health Questionnaire was completed by parents of 71 patients, while an additional 74 patients completed the Short Form--36. FD imposed a greater physical than psychosocial burden on the child, while the young adults reported both mental and physical quality of life within the average range. Self-esteem was problematic and improved with age, while both groups reported lowering physical quality of life as they grew older, with worsening general health that limited their role at school or work. Younger FD patients should be closely monitored for lowered self-esteem and referred for counseling when appropriate, while physical and occupational therapy should be provided in advance of expected lowered physical QoL and role fulfillment with increasing age. This becomes important as the need for additional surgical interventions, such as fundoplication with gastrostomy or spinal fusion, contribute to lower physical functioning. Given the high degree of parental involvement required for the varied manifestations of this multisystem disorder, the need for continued parental assessment and psycho-education about this chronic medical illness is warranted.

  9. Expanding subjectivities

    DEFF Research Database (Denmark)

    Lundgaard Andersen, Linda; Soldz, Stephen

    2012-01-01

    A major theme in recent psychoanalytic thinking concerns the use of therapist subjectivity, especially “countertransference,” in understanding patients. This thinking converges with and expands developments in qualitative research regarding the use of researcher subjectivity as a tool to understa...

  10. Cold pressor test demonstrates residual sympathetic cardiovascular activation in familial dysautonomia.

    Science.gov (United States)

    Hilz, M J; Axelrod, F B; Braeske, K; Stemper, B

    2002-04-15

    In familial dysautonomia (FD), i.e. Riley-Day-syndrome, sympathetic cardiovascular function, as well as afferent temperature and pain mediating neurons, are significantly reduced. Thus, it was questioned if cold pressor test (CPT), which normally enhances sympathetic outflow and induces peripheral vasoconstriction by the activation of thermo- and nociceptive system activation, could be used to assess sympathetic function in FD. To evaluate whether CPT can be used to assess sympathetic activation in FD, we performed CPT in 15 FD patients and 18 controls. After a 35-min resting period, participants immersed their right hand and arm up to the elbow into 0-1 degrees C cold water while we monitored heart rate (HR), respiration, beat-to-beat radial artery blood pressure (BP), and laser Doppler skin blood flow (SBF) at the right index finger pulp. From these measurements, heart rate variability parameters were calculated: root mean square of successive differences (RMSSD), coefficient of variation (CV), low and high frequency (LF, HF) power spectra of the electrocardiogram (ECG). All participants perceived cold stimulation and indicated discomfort. In controls, SBF decreased and HR and BP increased rapidly upon CPT. After 60 s, SBF indicated secondary vasodilatation in six controls, BP rise attenuated and HR returned to baseline in all controls. In the patients, SBF remained unchanged, HR and BP increased significantly, but after 50-60 s of CPT and changes were lower than in controls (p<0.05). RMSSD and CV decreased and LF increased significantly only in the controls. We conclude that CPT activates sympathetic HR and BP modulation despite impaired pain and temperature perception in FD patients. BP increase in the presence of almost unchanged SBF might be due to HR increase and to nociceptive arousal and emotionally induced catecholamine release as seen in emotional crises of FD patients. CPT assesses sympathetic cardiovascular responses independently from baroreflex

  11. Loss of Ikbkap Causes Slow, Progressive Retinal Degeneration in a Mouse Model of Familial Dysautonomia

    Science.gov (United States)

    Ramirez, Grisela

    2016-01-01

    Abstract Familial dysautonomia (FD) is an autosomal recessive congenital neuropathy that is caused by a mutation in the gene for inhibitor of kappa B kinase complex-associated protein (IKBKAP). Although FD patients suffer from multiple neuropathies, a major debilitation that affects their quality of life is progressive blindness. To determine the requirement for Ikbkap in the developing and adult retina, we generated Ikbkap conditional knockout (CKO) mice using a TUBA1a promoter-Cre (Tα1-Cre). In the retina, Tα1-Cre expression is detected predominantly in retinal ganglion cells (RGCs). At 6 months, significant loss of RGCs had occurred in the CKO retinas, with the greatest loss in the temporal retina, which is the same spatial phenotype observed in FD, Leber hereditary optic neuropathy, and dominant optic atrophy. Interestingly, the melanopsin-positive RGCs were resistant to degeneration. By 9 months, signs of photoreceptor degeneration were observed, which later progressed to panretinal degeneration, including RGC and photoreceptor loss, optic nerve thinning, Müller glial activation, and disruption of layers. Taking these results together, we conclude that although Ikbkap is not required for normal development of RGCs, its loss causes a slow, progressive RGC degeneration most severely in the temporal retina, which is later followed by indirect photoreceptor loss and complete retinal disorganization. This mouse model of FD is not only useful for identifying the mechanisms mediating retinal degeneration, but also provides a model system in which to attempt to test therapeutics that may mitigate the loss of vision in FD patients. PMID:27699209

  12. Fludrocortisone in patients with familial dysautonomia--assessing effect on clinical parameters and gene expression.

    Science.gov (United States)

    Axelrod, Felicia B; Goldberg, Judith D; Rolnitzky, Linda; Mull, James; Mann, Sandra P; Gold von Simson, Gabrielle; Berlin, Dena; Slaugenhaupt, Susan A

    2005-08-01

    The common familial dysautonomia (FD) mutation causes a splicing defect that leads to production of both wild-type (WT) and mutant (MU) IKBKAP mRNA. Because drugs may alter splicing, seven drugs, fludrocortisone, midodrine, diazepam, albuterol, clonidine, caffeine, and dopamine were screened. Since only fludrocortisone negatively altered gene expression, we assessed fludrocortisone's efficacy in treating postural hypotension, and its effect on survival and secondary long-term FD problems. For 341 FD patients we obtained demographic data and clinical information from the last Center evaluation (most current or prior to death) including mean blood pressures (supine, 1 min erect and 5 min erect) and history regarding syncope and presyncope symptoms. For 175 fludrocortisone-treated patients, data from the evaluation prior to start of fludrocortisone and from the last Center evaluation were compared. The fludrocortisone-treated patient cohort was compared to the nontreated patient cohort with respect to overall survival and event-free survival for crisis frequency, worsening gait, frequent fractures, spine curvature, renal insufficiency, and pacemaker insertion. Overall survivals of patients on fludrocortisone alone, on fludrocortisone and midodrine, and on neither drug were compared. Cumulative survival was significantly higher in fludrocortisone-treated patients than in non-treated patients during the first decade. In subsequent decades, the addition of midodrine improved cumulative survival. Fludrocortisone significantly increased mean blood pressures and decreased dizziness and leg cramping, but not headaches or syncope. Fludrocortisone was associated with more long-term problems, which may reflect more symptomatic status associated with longer survival. Our data suggest that fludrocortisone has clinical efficacy despite negative in vitro observations on gene expression.

  13. Molecular confirmation of t(6;11)(p21;q12) renal cell carcinoma in archival paraffin-embedded material using a break-apart TFEB FISH assay expands its clinicopathologic spectrum.

    Science.gov (United States)

    Argani, Pedram; Yonescu, Raluca; Morsberger, Laura; Morris, Kerry; Netto, George J; Smith, Nathan; Gonzalez, Nilda; Illei, Peter B; Ladanyi, Marc; Griffin, Constance A

    2012-10-01

    A subset of renal cell carcinomas (RCCs) is characterized by t(6;11)(p21;q12), which results in fusion of the untranslated Alpha (MALAT1) gene to the TFEB gene. Only 21 genetically confirmed cases of t(6;11) RCCs have been reported. This neoplasm typically demonstrates a distinctive biphasic morphology, comprising larger epithelioid cells and smaller cells clustered around basement membrane material; however, the full spectrum of its morphologic appearances is not known. The t(6;11) RCCs differ from most conventional RCCs in that they consistently express melanocytic immunohistochemical (IHC) markers such as HMB45 and Melan A and the cysteine protease cathepsin K but are often negative for epithelial markers such as cytokeratins. TFEB IHC has been proven to be useful to confirm the diagnosis of t(6;11) RCCs in archival material, because native TFEB is upregulated through promoter substitution by the gene fusion. However, IHC is highly fixation dependent and has been proven to be particularly difficult for TFEB. A validated fluorescence in situ hybridization (FISH) assay for molecular confirmation of the t(6;11) RCC in archival formalin-fixed, paraffin-embedded material has not been previously reported. We report herein the development of a break-apart TFEB FISH assay for the diagnosis of t(6;11)(p21;q12) RCCs. We validated the assay on 4 genetically confirmed cases and 76 relevant expected negative control cases and used the assay to report 8 new cases that expand the clinicopathologic spectrum of t(6;11) RCCs. An additional previously reported TFEB IHC-positive case was confirmed by TFEB FISH in 46-year-old archival material. In conclusion, TFEB FISH is a robust, clinically validated assay that can confirm the diagnosis of t(6;11) RCC in archival material and should allow a more comprehensive clinicopathologic delineation of this recently recognized neoplastic entity.

  14. Raine syndrome: expanding the radiological spectrum

    Energy Technology Data Exchange (ETDEWEB)

    Koob, Meriam; Dietemann, Jean-Louis [CHU de Strasbourg Hopital de Hautepierre, Service de Radiologie 2, Strasbourg (France); Doray, Berenice; Fradin, Melanie [CHU de Strasbourg, Hopital de Hautepierre, Laboratoire de Genetique Medicale, Strasbourg (France); Astruc, Dominique [CHU de Strasbourg Hopital de Hautepierre, Service de Neonatologie, Strasbourg (France)

    2011-03-15

    We describe ante- and postnatal imaging of a 1-year-old otherwise healthy girl with Raine syndrome. She presented with neonatal respiratory distress related to a pyriform aperture stenosis, which was diagnosed on CT. Signs of chondrodysplasia punctata, sagittal vertebral clefting and intervertebral disc and renal calcifications were also found on imaging. This new case confirms that Raine syndrome is not always lethal. The overlapping imaging signs with chondrodysplasia punctata and the disseminated calcifications give new insights into its pathophysiology. (orig.)

  15. Expanding Disease Spectrum Associated With Puerperal Mastitis

    Directory of Open Access Journals (Sweden)

    Gregg L. McAdoo

    1997-01-01

    Full Text Available Background: Staphylococcus aureus and the β-hemolytic streptococci are the commonest causes of puerperal mastitis which tends to be a localized disease process. This report describes 2 cases attributable to these bacteria that resulted in extramammary involvement and augmented morbidity.

  16. Genetics Home Reference: ataxia neuropathy spectrum

    Science.gov (United States)

    ... Understand Genetics Home Health Conditions ataxia neuropathy spectrum ataxia neuropathy spectrum Enable Javascript to view the expand/ ... boxes. Download PDF Open All Close All Description Ataxia neuropathy spectrum is part of a group of ...

  17. NT5C2 novel splicing variant expands the phenotypic spectrum of Spastic Paraplegia (SPG45): case report of a new member of thin corpus callosum SPG-Subgroup.

    Science.gov (United States)

    Elsaid, Mahmoud F; Ibrahim, Khalid; Chalhoub, Nader; Elsotouhy, Ahmed; El Mudehki, Noora; Abdel Aleem, Alice

    2017-03-21

    Hereditary Spastic Paraplegia (HSP) is a genetically heterogeneous group of neurodegenerative diseases. Thin Corpus Callosum (TCC) associated HSP is a distinguished subgroup of complex forms. Purines and pyrimidine, the basic DNA and RNA components, are regulating the cell metabolism, having roles in signal transduction, energy preservation and cellular repair. Genetic defects in nucleotide metabolism related genes have been only recently implicated in brain and neurodegenerative diseases' pathogenesis. We present a consanguineous Qatari family with two brothers, 9 and 3 years, who displayed a characteristic phenotype of early onset and markedly-severe spasticity with tiptoe walking, delayed dysarthric speech, persistent truncal hypotonia, and multiple variable-sized areas of brownish skin discoloration appearing at different places on the body. A clinical diagnosis suggestive of complex hereditary spastic paraplegia (HSP) was set after the family had the second affected child. Whole genome sequencing identified a novel homozygous NT5C2 splice site mutation (NM_012229.4/NM_001134373.2: c.1159 + 1G > T) that recessively segregated in family members. Brain MRI revealed dysgenic and thin corpus callosum (TCC) with peri-trigonal white matter cystic changes in both affected boys, whereas a well-developed corpus callosum with normal white matter was shown in their apparently normal brother, who found to be a carrier for the mutant variant. This mutation led to skipping of exon 14 with removal of 58 amino acid residues at the C-terminal half. The aberrantly spliced NT5C2 showed substantial reduction in expression level in the in-vitro study, indicating marked instability of the mutant NT5C2 protein. The present report expands the phenotypic spectrum of SPG45 and confirms NT5C2-SPG45 as a member of the rare TCC SPG-subtypes. Homozygous alteration in NT5C2 seems essential to produce central white matter developmental defects. The study highlights the importance of

  18. [A review of the oro-dento-facial characteristics of hereditary sensory and autonomic neuropathy type III (familial dysautonomia)].

    Science.gov (United States)

    Mass, E

    2013-01-01

    The oro-dento-facial features and dysfunction of children with hereditary sensory and autonomic neuropathy type Ill, known as familial dysautonomia or Riley-Day syndrome, was first described in the scientific literature in 1949. They include dental trauma; dental and soft tissue self-mutilation; normal dental age; normal sequence and timing of eruption and exfoliation of teeth; smaller tooth size; different and disproportional tooth components; normal alveolar bone height; small jaws, mild crowding, and malocclusions. These persons have craniofacial morphology that is different from accepted norms but they resemble norms of their ethnic origin. The subjects can have gray, pale, shiny faces with an asymmetric suffering expression; frontal bossing, with eventual hypertelorism and narrow lips; a low caries rate; drooling, and hypersalivation. They can have changes in salivary composition and content, which influences plaque and calculus and increases the risk of gingival and periodontal diseases. They also have difficulty in controlling oral muscles; a progressive decrease in number of tongue fungiform papillae, accompanied by lack of taste buds; and specific dysgeusia, but a normal sense of smell.

  19. A review of the oro-dento-facial characteristics of hereditary sensory and autonomic neuropathy type III (familial dysautonomia).

    Science.gov (United States)

    Mass, Eliyahu

    2012-01-01

    The oro-dento-facial features and dysfunction of children with hereditary sensory and autonomic neuropathy type III, known as familial dysautonomia or Riley-Day syndrome, was first described in the scientific literature in 1949. They include dental trauma; dental and soft tissue self-mutilation; normal dental age; normal sequence and timing of eruption and exfoliation of teeth; smaller tooth size; different and disproportional tooth components; normal alveolar bone height; small jaws, mild crowding, and malocclusions. These persons have craniofacial morphology that is different from accepted norms but they resemble norms of their ethnic origin. The subjects can have gray, pale, shiny faces with an asymmetric suffering expression; frontal bossing, with eventual hypertelorism and narrow lips; a low-caries rate; drooling, and hypersalivation. They can have changes in salivary composition and content, which influences plaque and calculus and increases the risk of gingival and periodontal diseases. They also have difficulty in controlling oral muscles; a progressive decrease in number of tongue fungiform papillae, accompanied by lack of taste buds; and specific dysgeusia, but a normal sense of smell.

  20. The familial dysautonomia disease gene IKBKAP is required in the developing and adult mouse central nervous system

    Directory of Open Access Journals (Sweden)

    Marta Chaverra

    2017-05-01

    Full Text Available Hereditary sensory and autonomic neuropathies (HSANs are a genetically and clinically diverse group of disorders defined by peripheral nervous system (PNS dysfunction. HSAN type III, known as familial dysautonomia (FD, results from a single base mutation in the gene IKBKAP that encodes a scaffolding unit (ELP1 for a multi-subunit complex known as Elongator. Since mutations in other Elongator subunits (ELP2 to ELP4 are associated with central nervous system (CNS disorders, the goal of this study was to investigate a potential requirement for Ikbkap in the CNS of mice. The sensory and autonomic pathophysiology of FD is fatal, with the majority of patients dying by age 40. While signs and pathology of FD have been noted in the CNS, the clinical and research focus has been on the sensory and autonomic dysfunction, and no genetic model studies have investigated the requirement for Ikbkap in the CNS. Here, we report, using a novel mouse line in which Ikbkap is deleted solely in the nervous system, that not only is Ikbkap widely expressed in the embryonic and adult CNS, but its deletion perturbs both the development of cortical neurons and their survival in adulthood. Primary cilia in embryonic cortical apical progenitors and motile cilia in adult ependymal cells are reduced in number and disorganized. Furthermore, we report that, in the adult CNS, both autonomic and non-autonomic neuronal populations require Ikbkap for survival, including spinal motor and cortical neurons. In addition, the mice developed kyphoscoliosis, an FD hallmark, indicating its neuropathic etiology. Ultimately, these perturbations manifest in a developmental and progressive neurodegenerative condition that includes impairments in learning and memory. Collectively, these data reveal an essential function for Ikbkap that extends beyond the peripheral nervous system to CNS development and function. With the identification of discrete CNS cell types and structures that depend on

  1. Scintigraphic differentiation between two forms of primary dysautonomia early after onset of autonomic dysfunction: value of cardiac and pulmonary iodine-123 MIBG uptake

    Energy Technology Data Exchange (ETDEWEB)

    Reinhardt, M.J.; Juengling, F.D.; Krause, T.M. [Dept. of Nuclear Medicine, Freiburg University Hospital (Germany); Braune, S. [Dept. of Neurology, Freiburg University Hospital (Germany)

    2000-05-01

    Primary dysfunction of the autonomic nervous system can be observed in patients with Parkinson's disease and those with multiple system atrophy. However, the fate of the two diseases differs considerably and leads to different strategies for patient management. Differentiation of the two diseases currently requires a combination of several clinical and electrophysiological tests. First studies of myocardial innervation using iodine-123 metaiodobenzylguanidine (MIBG) indicated a possible role of scintigraphy for this purpose. An increase in the pulmonary uptake of {sup 123}I-MIBG has been reported in secondary dysautonomias. Whether sympathetic innervation of the lung is affected in primary dysautonomias is currently unknown. Therefore, cardiac and pulmonary uptake of {sup 123}I-MIBG was studied in 21 patients with Parkinson's disease, 7 patients with multiple system atrophy and 13 age- and sex-matched controls. Thoracic images were obtained in the anterior view 4 h after intravenous injection of 185 MBq {sup 123}I-MIBG, at which time the maximum neuronal uptake is reached. All patients with Parkinson's disease had significantly lower cardiac uptake of {sup 123}I-MIBG than patients with multiple system atrophy and controls. Sympathetic innervation of the lung was not affected in either disease. It is concluded that scintigraphy with {sup 123}I-MIBG appears to be a useful tool for differentiation between Parkinson's disease and multiple system atrophy early after onset of autonomic dysfunction. (orig.)

  2. What Expands in an Expanding Universe?

    Science.gov (United States)

    Pacheco, José A De Freitas

    2015-01-01

    In the present investigation, the possible effects of the expansion of the Universe on systems bonded either by gravitational or electromagnetic forces, are reconsidered. It will be shown that the acceleration (positive or negative) of the expanding background, is the determinant factor affecting planetary orbits and atomic sizes. In the presently accepted cosmology (ΛCDM) all bonded systems are expanding at a decreasing rate that tends to be zero as the universe enters in a de Sitter phase. It is worth mentioning that the estimated expansion rates are rather small and they can be neglected for all practical purposes.

  3. What Expands in an Expanding Universe?

    Directory of Open Access Journals (Sweden)

    JOSÉ A. DE FREITAS PACHECO

    2015-12-01

    Full Text Available ABSTRACT In the present investigation, the possible effects of the expansion of the Universe on systems bonded either by gravitational or electromagnetic forces, are reconsidered. It will be shown that the acceleration (positive or negative of the expanding background, is the determinant factor affecting planetary orbits and atomic sizes. In the presently accepted cosmology (ΛCDM all bonded systems are expanding at a decreasing rate that tends to be zero as the universe enters in a de Sitter phase. It is worth mentioning that the estimated expansion rates are rather small and they can be neglected for all practical purposes.

  4. Disautonomia: um conceito emergente na síndrome da fibromialgia Dysautonomia: an emerging concept in fibromyalgia syndrome

    Directory of Open Access Journals (Sweden)

    Luiza Cristina Lacerda Jacomini

    2007-10-01

    chronic, widespread pain condition that has important prevalence in general population. Despite the musculoskeletal pain, the majority of people with fibromyalgia also experience fatigue, sleep disorders, visceral pain, exercise intolerance and neurological symptoms. This syndrome is considered a functional syndrome because it is better characterized by its symptoms, suffering and disability rather than well seen organic structure impairment. A substantial literature has been produced in order to explain the pathophysiology of fibromyalgia: researches on genetics, biogenic amines, neurotransmitters, hypothalamic-pituitary-adrenal axis hormones, oxidative stress, mechanisms of pain modulation, central sensitization and autonomic function in FM revealed various abnormalities indicating that multiple factors and mechanisms may be involved in the pathogenesis of FM. Dysautonomia may have a main role at FM pathophysiology, even though it is not clear if cause, consequence or a component of this mosaic syndrome. Future research must be encouraged in order to evaluate the real role of autonomic nervous system or its impaired function in the complex pathophysiology of the syndrome and in order to assess efficacy of therapeutics interventions through well designed assays. The development of effective therapeutics measures has been delayed due to lack of knowledge of the main mechanisms in the pathogenesis of FM. A thorough understanding of mechanisms will be required to develop and document convenient therapies. This review aims to describe the most recent articles that document autonomic system impairment in SFM and to discuss dysautonomia as a potential mechanism that plays a role in the symptoms genesis, symptoms maintenance and comorbidity in SFM.

  5. Dysautonomia (Autonomic Dysfunction)

    Science.gov (United States)

    ... It can be acute and reversible, as in Guillain-Barre syndrome, or chronic and progressive. Several common conditions ... It can be acute and reversible, as in Guillain-Barre syndrome, or chronic and progressive. Several common conditions ...

  6. Forehead tissue expander.

    Science.gov (United States)

    Kisner, W H

    1991-02-01

    The use of the forehead flap for nasal reconstruction has long been used by reconstructive surgeons. A case is presented in which comprised forehead skin is utilized following expansion by a tissue expander.

  7. Expanding the clinicopathological spectrum of late cutaneous Lyme borreliosis (acrodermatitis chronica atrophicans [ACA]): A prospective study of 20 culture- and/or polymerase chain reaction (PCR)-documented cases.

    Science.gov (United States)

    Lenormand, Cédric; Jaulhac, Benoît; Debarbieux, Sébastien; Dupin, Nicolas; Granel-Brocard, Florence; Adamski, Henri; Barthel, Cathy; Cribier, Bernard; Lipsker, Dan

    2016-04-01

    The diagnosis of acrodermatitis chronica atrophicans (ACA), the late cutaneous manifestation of Lyme borreliosis, can be challenging. Histologic changes in ACA have been described in a few studies from endemic countries, relying on cases documented by serology only. We sought to reassess the clinicopathological spectrum of ACA in a series of thoroughly documented cases. Patients prospectively included in a national prospective study were selected on the basis of positive culture and/or polymerase chain reaction of a skin biopsy sample. The diagnosis of ACA was confirmed by reviewing the clinical and serologic data. Histopathological samples were carefully reviewed. Twenty patients were included. Unusual clinical features (ie, numerous small violaceous patches and equidistant small spinous papules with background faint erythema) were observed in 2 patients. Histopathological examination revealed a classic plasma cell-rich perivascular and interstitial pattern with telangiectases in 16 of 25 samples, whereas strikingly prominent granuloma annulare-like or lichenoid features were observed in 4 and 2 of 25 cases, respectively, and discrete nonspecific minor changes in 3 of 25 cases. The small number of patients was a limitation. Genuine culture- and/or polymerase chain reaction-proven ACA can rarely present as numerous violaceous patches or cluster of spinous papules clinically, and as a granuloma annulare-like or lichenoid dermatosis histologically. Copyright © 2015 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  8. Non-length-dependent and length-dependent small-fiber neuropathies associated with tumor necrosis factor (TNF)-inhibitor therapy in patients with rheumatoid arthritis: expanding the spectrum of neurological disease associated with TNF-inhibitors.

    Science.gov (United States)

    Birnbaum, Julius; Bingham, Clifton O

    2014-04-01

    Small-fiber neuropathy causes severe burning pain, requires diagnostic approaches such as skin biopsy, and encompasses two subtypes based on distribution of neuropathic pain. Such biopsy-proven subtypes of small-fiber neuropathies have not been previously described as complications of tumor necrosis factor (TNF)-inhibitor therapy. We therefore characterized clinical and skin biopsy findings in three rheumatoid arthritis (RA) patients who developed small-fiber neuropathies associated with TNF-inhibitors. We also conducted a systematic review of the literature to characterize subtypes of neuropathies previously reported in association with TNF-inhibitor therapy. Two patients presented with a "non-length-dependent" small-fiber neuropathy, experiencing unorthodox patterns of burning pain affecting the face, torso, and proximal extremities. Abnormal skin biopsy findings were limited to the proximal thigh, which is a marker of proximal-most dorsal root ganglia degeneration. In contrast, one patient presented with a "length-dependent" small-fiber neuropathy, experiencing burning pain only in the feet. Abnormal skin biopsy findings were limited to the distal feet, which is a marker of distal-most axonal degeneration. One patient developed a small-fiber neuropathy in the context of TNF-inhibitor-induced lupus. In all patients, neuropathies occurred during TNF-inhibitor-induced remission of RA disease activity and improved on withdrawal of TNF-inhibitors. We describe a spectrum of small-fiber neuropathies not previously reported in association with TNF-inhibitor therapy, with clinical and skin biopsy findings suggestive of dorsal root ganglia as well as axonal degeneration. The development of small-fiber neuropathies during inactive joint disease and improvement of neuropathic pain upon withdrawal of TNF-inhibitor suggest a causative role of TNF-inhibitors. © 2013 Published by Elsevier Inc.

  9. On Expanded Cyclic Codes

    CERN Document Server

    Wu, Yingquan

    2008-01-01

    The paper has a threefold purpose. The first purpose is to present an explicit description of expanded cyclic codes defined in $\\GF(q^m)$. The proposed explicit construction of expanded generator matrix and expanded parity check matrix maintains the symbol-wise algebraic structure and thus keeps many important original characteristics. The second purpose of this paper is to identify a class of constant-weight cyclic codes. Specifically, we show that a well-known class of $q$-ary BCH codes excluding the all-zero codeword are constant-weight cyclic codes. Moreover, we show this class of codes achieve the Plotkin bound. The last purpose of the paper is to characterize expanded cyclic codes utilizing the proposed expanded generator matrix and parity check matrix. We analyze the properties of component codewords of a codeword and particularly establish the precise conditions under which a codeword can be represented by a subbasis. With the new insights, we present an improved lower bound on the minimum distance of...

  10. Silicon microfabricated beam expander

    Science.gov (United States)

    Othman, A.; Ibrahim, M. N.; Hamzah, I. H.; Sulaiman, A. A.; Ain, M. F.

    2015-03-01

    The feasibility design and development methods of silicon microfabricated beam expander are described. Silicon bulk micromachining fabrication technology is used in producing features of the structure. A high-precision complex 3-D shape of the expander can be formed by exploiting the predictable anisotropic wet etching characteristics of single-crystal silicon in aqueous Potassium-Hydroxide (KOH) solution. The beam-expander consist of two elements, a micromachined silicon reflector chamber and micro-Fresnel zone plate. The micro-Fresnel element is patterned using lithographic methods. The reflector chamber element has a depth of 40 µm, a diameter of 15 mm and gold-coated surfaces. The impact on the depth, diameter of the chamber and absorption for improved performance are discussed.

  11. Silicon microfabricated beam expander

    Energy Technology Data Exchange (ETDEWEB)

    Othman, A., E-mail: aliman@ppinang.uitm.edu.my; Ibrahim, M. N.; Hamzah, I. H.; Sulaiman, A. A. [Faculty of Electrical Engineering, Universiti Teknologi MARA Malaysia, 40450, Shah Alam, Selangor (Malaysia); Ain, M. F. [School of Electrical and Electronic Engineering, Engineering Campus, Universiti Sains Malaysia, Seri Ampangan, 14300,Nibong Tebal, Pulau Pinang (Malaysia)

    2015-03-30

    The feasibility design and development methods of silicon microfabricated beam expander are described. Silicon bulk micromachining fabrication technology is used in producing features of the structure. A high-precision complex 3-D shape of the expander can be formed by exploiting the predictable anisotropic wet etching characteristics of single-crystal silicon in aqueous Potassium-Hydroxide (KOH) solution. The beam-expander consist of two elements, a micromachined silicon reflector chamber and micro-Fresnel zone plate. The micro-Fresnel element is patterned using lithographic methods. The reflector chamber element has a depth of 40 µm, a diameter of 15 mm and gold-coated surfaces. The impact on the depth, diameter of the chamber and absorption for improved performance are discussed.

  12. Expanding mediation theory

    NARCIS (Netherlands)

    Verbeek, P.P.C.C.

    2012-01-01

    In his article In Between Us, Yoni van den Eede expands existing theories of mediation into the realm of the social and the political, focusing on the notions of opacity and transparency. His approach is rich and promising, but two pitfalls should be avoided. First, his concept of ‘in-between’ runs

  13. Expanding Student Assessment Opportunities.

    Science.gov (United States)

    Bartscher, Beth; Carter, Andrea; Lawlor, Anna; McKelvey, Barbara

    This paper describes an approach for expanding assessment opportunities for students to demonstrate their understanding of content. The targeted population consisted of elementary and junior high school students in two schools in a growing middle-class community in north central Illinois. The elementary school enrolled 467 students and the junior…

  14. Grazing incidence beam expander

    Energy Technology Data Exchange (ETDEWEB)

    Akkapeddi, P.R.; Glenn, P.; Fuschetto, A.; Appert, Q.; Viswanathan, V.K.

    1985-01-01

    A Grazing Incidence Beam Expander (GIBE) telescope is being designed and fabricated to be used as an equivalent end mirror in a long laser resonator cavity. The design requirements for this GIBE flow down from a generic Free Electron Laser (FEL) resonator. The nature of the FEL gain volume (a thin, pencil-like, on-axis region) dictates that the output beam be very small. Such a thin beam with the high power levels characteristic of FELs would have to travel perhaps hundreds of meters or more before expanding enough to allow reflection from cooled mirrors. A GIBE, on the other hand, would allow placing these optics closer to the gain region and thus reduces the cavity lengths substantially. Results are presented relating to optical and mechanical design, alignment sensitivity analysis, radius of curvature analysis, laser cavity stability analysis of a linear stable concentric laser cavity with a GIBE. Fabrication details of the GIBE are also given.

  15. IKAP/hELP1 deficiency in the cerebrum of familial dysautonomia patients results in down regulation of genes involved in oligodendrocyte differentiation and in myelination.

    Science.gov (United States)

    Cheishvili, David; Maayan, Channa; Smith, Yoav; Ast, Gil; Razin, Aharon

    2007-09-01

    The gene affected in the congenital neuropathy familial dysautonomia (FD) is IKBKAP that codes for the IKAP/hELP1 protein. Several different functions have been suggested for this protein, but none of them have been verified in vivo or shown to have some link with the FD phenotype. In an attempt to elucidate the involvement of IKAP/hELP1 in brain function, we searched for IKAP/hELP1 target genes associated with neuronal function. In a microarray expression analysis using RNA extracted from the cerebrum of two FD patients as well as sex and age matched controls, no genes were found to be upregulated in the FD cerebrum. However, 25 genes were downregulated more than 2-fold in the cerebrum of both the male FD child and female FD mature woman. Thirteen of them are known to be involved in oligodendrocyte development and myelin formation. The down regulation of all these genes was verified by real-time PCR. Four of these genes were also confirmed to be downregulated at the protein level. These results are statistically significant and have high biological relevance, since seven of the downregulated genes in the cerebrum of the FD patients were shown by others to be upregulated during oligodendrocyte differentiation in vitro. Our results therefore suggest that IKAP/hELP1 may play a role in oligodendrocyte differentiation and/or myelin formation.

  16. Familial Dysautonomia (FD Human Embryonic Stem Cell Derived PNS Neurons Reveal that Synaptic Vesicular and Neuronal Transport Genes Are Directly or Indirectly Affected by IKBKAP Downregulation.

    Directory of Open Access Journals (Sweden)

    Sharon Lefler

    Full Text Available A splicing mutation in the IKBKAP gene causes Familial Dysautonomia (FD, affecting the IKAP protein expression levels and proper development and function of the peripheral nervous system (PNS. Here we found new molecular insights for the IKAP role and the impact of the FD mutation in the human PNS lineage by using a novel and unique human embryonic stem cell (hESC line homozygous to the FD mutation originated by pre implantation genetic diagnosis (PGD analysis. We found that IKBKAP downregulation during PNS differentiation affects normal migration in FD-hESC derived neural crest cells (NCC while at later stages the PNS neurons show reduced intracellular colocalization between vesicular proteins and IKAP. Comparative wide transcriptome analysis of FD and WT hESC-derived neurons together with the analysis of human brains from FD and WT 12 weeks old embryos and experimental validation of the results confirmed that synaptic vesicular and neuronal transport genes are directly or indirectly affected by IKBKAP downregulation in FD neurons. Moreover we show that kinetin (a drug that corrects IKBKAP alternative splicing promotes the recovery of IKAP expression and these IKAP functional associated genes identified in the study. Altogether, these results support the view that IKAP might be a vesicular like protein that might be involved in neuronal transport in hESC derived PNS neurons. This function seems to be mostly affected in FD-hESC derived PNS neurons probably reflecting some PNS neuronal dysfunction observed in FD.

  17. Familial Dysautonomia (FD) Human Embryonic Stem Cell Derived PNS Neurons Reveal that Synaptic Vesicular and Neuronal Transport Genes Are Directly or Indirectly Affected by IKBKAP Downregulation.

    Science.gov (United States)

    Lefler, Sharon; Cohen, Malkiel A; Kantor, Gal; Cheishvili, David; Even, Aviel; Birger, Anastasya; Turetsky, Tikva; Gil, Yaniv; Even-Ram, Sharona; Aizenman, Einat; Bashir, Nibal; Maayan, Channa; Razin, Aharon; Reubinoff, Benjamim E; Weil, Miguel

    2015-01-01

    A splicing mutation in the IKBKAP gene causes Familial Dysautonomia (FD), affecting the IKAP protein expression levels and proper development and function of the peripheral nervous system (PNS). Here we found new molecular insights for the IKAP role and the impact of the FD mutation in the human PNS lineage by using a novel and unique human embryonic stem cell (hESC) line homozygous to the FD mutation originated by pre implantation genetic diagnosis (PGD) analysis. We found that IKBKAP downregulation during PNS differentiation affects normal migration in FD-hESC derived neural crest cells (NCC) while at later stages the PNS neurons show reduced intracellular colocalization between vesicular proteins and IKAP. Comparative wide transcriptome analysis of FD and WT hESC-derived neurons together with the analysis of human brains from FD and WT 12 weeks old embryos and experimental validation of the results confirmed that synaptic vesicular and neuronal transport genes are directly or indirectly affected by IKBKAP downregulation in FD neurons. Moreover we show that kinetin (a drug that corrects IKBKAP alternative splicing) promotes the recovery of IKAP expression and these IKAP functional associated genes identified in the study. Altogether, these results support the view that IKAP might be a vesicular like protein that might be involved in neuronal transport in hESC derived PNS neurons. This function seems to be mostly affected in FD-hESC derived PNS neurons probably reflecting some PNS neuronal dysfunction observed in FD.

  18. 通过双色场和静电场的合成来拓宽高次谐波谱%Expanding the high-order harmonic spectrum by using the combination of a two-color field and a static electric field

    Institute of Scientific and Technical Information of China (English)

    吕婷婷; 都慧妮; 苗向阳

    2012-01-01

    An efficient method to extend the plateau of high-order harmonic spectrum by using the combination of a two-color laser field and a static electric field is presented. We numerically solve one-dimensional time-dependent Schrodinge equation of helium atom and the high-order harmonic spectrum in different synthesized field can be obtained, meanwhile, analyzing the influence of static electric field on high-order harmonic generation (HHG) with classical returning kinetic-energy map and time-frequency distribution. The results show that, by adding a static electric field to a synthesized two-color laser field, the width of the plateau can be expanded efficiently, and a bandwidth of nearly 358eV can be received.%本篇文章提出了一种通过双色激光场和静电场合成来拓宽高次谐波谱平台区域的方案.我们针对氦原子数值求解了一维含时薛定谔方程,并且得到了不同合成激光场条件下的高次谐波谱,并结合了经典电离-回碰动能分布图和时频分布图分析了静电场对高次谐波谱产生过程的影响.计算结果表明,通过在双色激光场上叠加一个静电场,可以有效地拓宽高次谐波谱的平台区域,从而得到一个频带宽度为358 eV的超连续谱.

  19. Expanding the HAWC Observatory

    Energy Technology Data Exchange (ETDEWEB)

    Mori, Johanna [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2016-08-17

    The High Altitude Water Cherenkov Gamma-Ray Observatory is expanding its current array of 300 water tanks to include 350 outrigger tanks to increase sensitivity to gamma rays above 10 TeV. This involves creating and testing hardware with which to build the new tanks, including photomultiplier tubes, high voltage supply units, and flash analog to digital converters. My responsibilities this summer included preparing, testing and calibrating that equipment.

  20. Antiphospholipid Syndrome: Expanding the Spectrum of Autoimmune Thrombosis

    OpenAIRE

    Gómez Puerta, José Alfredo

    2007-01-01

    "SINDROME ANTIFOSFOLIPIDICO: EXPANDIENDO EL ESPECTRO CLÍNICA DE LA TROMBOSIS AUTOINMUNE"El síndrome antifosfolipídico (SAF) es un síndrome protrombótico adquirido caracterizado por trombosis venosas y arteriales y pérdidas fetales recurrentes. Puede estar presente como SAF "primario" cuando no esta asociado a ninguna enfermedad autoinmune [fundamentalmente el lupus eritematoso sistémico (LES)] o en asociación a otros procesos tales como infecciones y procesos neoplásicos, entre otros. También...

  1. Antiphospholipid Syndrome: Expanding the Spectrum of Autoimmune Thrombosis

    OpenAIRE

    Gómez Puerta, José Alfredo

    2007-01-01

    "SINDROME ANTIFOSFOLIPIDICO: EXPANDIENDO EL ESPECTRO CLÍNICA DE LA TROMBOSIS AUTOINMUNE"El síndrome antifosfolipídico (SAF) es un síndrome protrombótico adquirido caracterizado por trombosis venosas y arteriales y pérdidas fetales recurrentes. Puede estar presente como SAF "primario" cuando no esta asociado a ninguna enfermedad autoinmune [fundamentalmente el lupus eritematoso sistémico (LES)] o en asociación a otros procesos tales como infecciones y procesos neoplásicos, entre otros. También...

  2. From WMD to WME: An Ever-Expanding Threat Spectrum

    Directory of Open Access Journals (Sweden)

    Bowman H. Miller

    2015-10-01

    Full Text Available One of the challenges the United States and its intelligence community confronts today, if not the foremost challenge, is the girth of its national security problem set. The array of threat types, as well as the potential sources of those threats, is unprecedented and growing. The burdensome task for intelligence at all times, but especially given the present rate of change and the increasing porosity of borders, is to try to cope with an escalating mix of challenges and rising expectations of what intelligence can provide. Existing tasks persist; they are not replaced. The number and types of potentially threatening actors have exploded. Nation-states are now joined by countless ethno-religious groupings, terrorists, criminals of all stripes, drug cartels, transnational movements and issue groups, and malevolent and delinquent individuals. Threats come from all quarters and in all sizes these days, and the mission of intelligence, i.e., to track indicators to provide warning and to reduce uncertainty for decision-makers, is monumental.

  3. Osteoma of long bone: an expanding spectrum of imaging findings.

    Science.gov (United States)

    Hansford, Barry Glenn; Pytel, Peter; Moore, Drew D; Stacy, Gregory Scott

    2015-05-01

    Osteoma of long bone is an extremely rare, benign bone-forming surface lesion with the largest published case series consisting of only 14 patients. The most important and often most difficult lesion to differentiate from osteoma of long bone radiographically is parosteal osteosarcoma, which is a rare, low-grade surface osteosarcoma with the potential for dedifferentiation. Reports of imaging studies of osteoma of long bone depict a well-defined ossified mass arising from the surface of the diaphysis or metadiaphysis of a long bone. A characteristic feature is the homogeneity of the mass, with uniform density near or equal to that of cortical bone from the base of the lesion to its periphery. The 45-year-old female in this case presented with left hip fullness and was subsequently found to have a proximal femoral osteoma, which was unique in that it contained large fatty marrow spaces that corresponded to bands of relatively low density on plain radiography and computed tomography, giving it a heterogeneous appearance atypical of osteoma of long bone. Furthermore, the osteoma reported here was associated with a small but separate nodular focus of ossification in the adjacent soft tissue. These findings led to a presumptive diagnosis of parosteal osteosarcoma with a local soft tissue metastasis or satellite nodule resulting in radical resection of the tumor. Definitive diagnosis of osteoma was made on histology of both the parent lesion and ossified nodule as no neoplastic spindle cell proliferation was present to establish a diagnosis of low-grade osteosarcoma. This represents, to the best of our knowledge, the first such presentation of osteoma of long bone.

  4. Expanding Your Horizon 2015

    CERN Multimedia

    Kaltenhauser, Kristin

    2015-01-01

    Expanding your horizons is a bi-annual “Science Day” for girls aged 11 to 14, held at the University of Geneva on 14 November. The girls had the opportunity to take part in hands-on workshops held by local professional women in the field of science, mathematics, engineering and technology. For the fourth time, CERN was part of this event, offering three workshops as well as a booth at the Discovery Fair, including Higgnite, an interactive visualization of the Higgs Field.

  5. The expanding universe

    CERN Document Server

    Lew, Kristi

    2011-01-01

    People have always been fascinated with the stars above and the universe that contains them. Over the years, astronomers have developed numerous theories to explain how the universe began, how it works, and what its ultimate fate will be. But all of the scientists' questions are far from answered. The Expanding Universe goes beyond the creation of the universe to explain how scientists think the universe works, grows, and changes, including what great thinkers Isaac Newton and Albert Einstein had to say about its fate. Readers will also learn about how researchers are slowly shedding light on

  6. Consonant confusions in amplitude-expanded speech.

    Science.gov (United States)

    Freyman, R L; Nerbonne, G P

    1996-12-01

    The perceptual consequences of expanding the amplitude variations in speech were studied under conditions in which spectral information was obscured by signal correlated noise that had an envelope correlated with the speech envelope, but had a flat amplitude spectrum. The noise samples, created individually from 22 vowel-consonant-vowel nonsense words, were used as maskers of those words, with signal-to-noise ratios ranging from -15 to 0 dB. Amplitude expansion was by a factor of 3.0 in terms of decibels. In the first experiment, presentation level for speech peaks was 80 dB SPL. Consonant recognition performance for expanded speech by 50 listeners with normal hearing was as much as 30 percentage points poorer than for unexpanded speech and the types of errors were dramatically different, especially in the midrange of S-N ratios. In a second experiment presentation level was varied to determine whether reductions in consonant levels produced by expansion were responsible for the differences between conditions. Recognition performance for unexpanded speech at 40 dB SPL was nearly equivalent to that for expanded speech at 80 dB SPL. The error patterns obtained in these two conditions were different, suggesting that the differences between conditions in Experiment 1 were due largely to expanded amplitude envelopes rather than differences in audibility.

  7. Deletion of exon 20 of the Familial Dysautonomia gene Ikbkap in mice causes developmental delay, cardiovascular defects, and early embryonic lethality.

    Directory of Open Access Journals (Sweden)

    Paula Dietrich

    Full Text Available Familial Dysautonomia (FD is an autosomal recessive disorder that affects 1/3,600 live births in the Ashkenazi Jewish population, and leads to death before the age of 40. The disease is characterized by abnormal development and progressive degeneration of the sensory and autonomic nervous system. A single base pair substitution in intron 20 of the Ikbkap gene accounts for 98% of FD cases, and results in the expression of low levels of the full-length mRNA with simultaneous expression of an aberrantly spliced mRNA in which exon 20 is missing. To date, there is no animal model for the disease, and the essential cellular functions of IKAP--the protein encoded by Ikbkap--remain unknown. To better understand the normal function of IKAP and in an effort to generate a mouse model for FD, we have targeted the mouse Ikbkap gene by homologous recombination. We created two distinct alleles that result in either loss of Ikbkap expression, or expression of an mRNA lacking only exon 20. Homozygosity for either mutation leads to developmental delay, cardiovascular and brain malformations, accompanied with early embryonic lethality. Our analyses indicate that IKAP is essential for expression of specific genes involved in cardiac morphogenesis, and that cardiac failure is the likely cause of abnormal vascular development and embryonic lethality. Our results also indicate that deletion of exon 20 abolishes gene function. This implies that the truncated IKAP protein expressed in FD patients does not retain any significant biological function.

  8. Massive Fermi gas in the expanding universe

    Science.gov (United States)

    Trautner, Andreas

    2017-03-01

    The behavior of a decoupled ideal Fermi gas in a homogeneously expanding three-dimensional volume is investigated, starting from an equilibrium spectrum. In case the gas is massless and/or completely degenerate, the spectrum of the gas can be described by an effective temperature and/or an effective chemical potential, both of which scale down with the volume expansion. In contrast, the spectrum of a decoupled massive and non-degenerate gas can only be described by an effective temperature if there are strong enough self-interactions such as to maintain an equilibrium distribution. Assuming perpetual equilibration, we study a decoupled gas which is relativistic at decoupling and then is red-shifted until it becomes non-relativistic. We find expressions for the effective temperature and effective chemical potential which allow us to calculate the final spectrum for arbitrary initial conditions. This calculation is enabled by a new expansion of the Fermi-Dirac integral, which is for our purpose superior to the well-known Sommerfeld expansion. We also compute the behavior of the phase space density under expansion and compare it to the case of real temperature and real chemical potential. Using our results for the degenerate case, we also obtain the mean relic velocity of the recently proposed non-thermal cosmic neutrino background.

  9. The Artful Universe Expanded

    Energy Technology Data Exchange (ETDEWEB)

    Bassett, B A [Institute of Cosmology and Gravitation, University of Portsmouth (United Kingdom)

    2005-07-29

    The cosmos is an awfully big place and there is no better guide to its vast expanse and fascinating nooks and crannies than John Barrow. A professor of mathematical sciences at Cambridge University, Barrow embodies that rare combination of highly polished writer and expert scientist. His deft touch brings together the disparate threads of human knowledge and weaves them into a tapestry as rich and interesting for the expert as it is for the layperson. The Artful Universe Expanded is an updated edition of this popular book first published in 1995. It explores the deeply profound manner in which natural law and the nature of the cosmos have moulded and shaped us, our cultures and the very form of our arts and music-a new type of 'cosmic' anthropology. The main themes Barrow chooses for revealing this new anthropology are the subjects of evolution, the size of things, the heavens and the nature of music. The book is a large, eclectic repository of knowledge often unavailable to the layperson, hidden in esoteric libraries around the world. It rivals The Da Vinci Code for entertainment value and insights, but this time it is Nature's code that is revealed. It is rare indeed to find common threads drawn through topics as diverse as The Beetles, Bach and Beethoven or between Jackson Pollock, the Aztecs, Kant, Picasso, Byzantine mosaics, uranium-235 and the helix nebula. Barrow unerringly binds them together, presenting them in a stimulating, conversational style that belies the amount of time that must have gone into researching this book. Dip into it at random, or read it from cover to cover, but do read it. The Artful Universe Expanded is an entertaining antidote to the oft-lamented pressures to know more and more about less and less and the apparently inexorable march of specialization. On reading this book one can, for a short time at least, hold in one's mind a vision that unifies science, art and culture and glimpse a universal tapestry of great

  10. Distribuição volumétrica e espectro de gotas de bicos hidráulicos de jato plano de faixa expandida XR11003 Volumetric distribution and spectrum of drops of hidraulic nozzles of plane jet of expanded strip XR11003

    Directory of Open Access Journals (Sweden)

    Felipe T. da Camara

    2008-12-01

    Full Text Available O presente trabalho teve por objetivos avaliar o espectro de gotas e os perfis de distribuição volumétrica de um bico hidráulico de jato plano de faixa expandida, modelo XR11003. Utilizou-se de analisador de partículas a laser para avaliar o espectro de gotas e de mesa de deposição para análise da distribuição volumétrica. O ensaio do espectro de gotas foi em delineamento inteiramente casualizado, em esquema fatorial 2 x 2 x 3, com três repetições, em que o primeiro fator representa o líquido pulverizado (L1 = água e L2 = água + 0,1% de espalhante adesivo não-iônico, o segundo representa a pressão de pulverização (P1 = 200 kPa e P2 = 400 kPa e o terceiro representa três bicos hidráulicos XR11003 de jato plano (B1, B2 e B3. No ensaio de distribuição volumétrica, o delineamento foi o inteiramente casualizado, em esquema fatorial com dois fatores, não se avaliando o fator bico. Dos resultados do espectro de gotas, observaram-se maior tamanho de gotas para a pressão de 200 kPa e menor amplitude relativa quando se utilizou 0,1% de adjuvante. Para os perfis de distribuição volumétrica, ocorreu aumento na faixa de deposição e no espaçamento entre bicos com C.V. de 10%, com a adição de 0,1% de adjuvante e aumento na pressão.The present work had for objective to evaluate the spectrum of drops and the volumetric distribution of a hydraulic nozzle of a plane jet of expanded strip, model XR11003. A laser particle analyzer was used to evaluate the spectrum of drops and a deposition table was used for analysis of the volumetric distribution. The experiment of the spectrum of drops was a completely randomized design, factorial 2 x 2 x 3, with three repetitions, where the first factor represents the sprayed liquid (L1 = water and L2 = water + 0,1% of no-ionic surfactant, the second factor represents the application pressure (P1 = 200 kPa e P2 = 400 kPa and the third factor represents three nozzles XR11003 of the plane jet

  11. Expanding contraceptive options.

    Science.gov (United States)

    1989-01-01

    The goals of Family Health International (FHI) have been to introduce a variety of birth control options to people in developing countries, and to provide information to the user on the advantages and disadvantages of each method. FHI has worked with many developing countries in clinical trials of established as well as new contraceptive methods. These trials played an important part in making 2 sterilization procedures, laparoscopy and minilaparotomy popular for women. Further research improved the methods and have made them the most popular in the world, chosen by 130 million users. FHI is doing clinical trials on a new IUD, that is a copper bearing T-shaped device called the TCu380A. they have collected data on over 10,000 women using IUD's and early analysis indicates TCu380A is more effective than others. FHI is also evaluating devices such as Norplant that will prevent pregnancy up to 5 years by implanting the capsules in the arm. More than 8,000 women are being tested to determine the acceptability of implants in different geographical locations. Other research groups are doing work in 10 additional countries: Bangladesh will expand its program to 24,000 women and Nepal to 8,000 women. Trials are also being conducted on progestogen pills, since they do not lesson the volume of milk in breast feeding. FHI has also worked to introduce creative community-based distribution channels. In one case, specially trained health workers delivered contraceptives door-to-door in over 150,000 households. They found that 2 of 3 women accepted the pills and in a follow up survey 90% were still using them. FHI is now focusing on ways to improve moving new contraceptives from clinical testing on everyday use. They will coordinate training programs, educational material, media campaigns, and efforts with other international organizations, government agencies, and family planning groups.

  12. The Artful Universe Expanded

    Science.gov (United States)

    Barrow, John D.

    2005-07-01

    Our love of art, writes John Barrow, is the end product of millions of years of evolution. How we react to a beautiful painting or symphony draws upon instincts laid down long before humans existed. Now, in this enhanced edition of the highly popular The Artful Universe , Barrow further explores the close ties between our aesthetic appreciation and the basic nature of the Universe. Barrow argues that the laws of the Universe have imprinted themselves upon our thoughts and actions in subtle and unexpected ways. Why do we like certain types of art or music? What games and puzzles do we find challenging? Why do so many myths and legends have common elements? In this eclectic and entertaining survey, Barrow answers these questions and more as he explains how the landscape of the Universe has influenced the development of philosophy and mythology, and how millions of years of evolutionary history have fashioned our attraction to certain patterns of sound and color. Barrow casts the story of human creativity and thought in a fascinating light, considering such diverse topics as our instinct for language, the origins and uses of color in nature, why we divide time into intervals as we do, the sources of our appreciation of landscape painting, and whether computer-generated fractal art is really art. Drawing on a wide variety of examples, from the theological questions raised by St. Augustine and C.S. Lewis to the relationship between the pure math of Pythagoras and the music of the Beatles, The Artful Universe Expanded covers new ground and enters a wide-ranging debate about the meaning and significance of the links between art and science.

  13. Zellweger Spectrum

    Science.gov (United States)

    ... severe defect, resulting in essentially nonfunctional peroxisomes. This phenomenon produces the range of severity of the disorders. How is the Zellweger Spectrum Diagnosed? The distinctive shape of the head and face of a child born with one of the diseases of the ...

  14. Trivalent expanders and hyperbolic surfaces

    CERN Document Server

    Ivrissimtzis, Ioannis; Vdovina, Alina

    2012-01-01

    We introduce a family of trivalent expanders which tessellate compact hyperbolic surfaces with large isometry groups. We compare this family with Platonic graphs and modifications of them and prove topological and spectral properties of these families.

  15. Fission Spectrum

    Science.gov (United States)

    Bloch, F.; Staub, H.

    1943-08-18

    Measurements of the spectrum of the fission neutrons of 25 are described, in which the energy of the neutrons is determined from the ionization produced by individual hydrogen recoils. The slow neutrons producing fission are obtained by slowing down the fast neutrons from the Be-D reaction of the Stanford cyclotron. In order to distinguish between fission neutrons and the remaining fast cyclotron neutrons both the cyclotron current and the pusle amplifier are modulated. A hollow neutron container, in which slow neutrons have a lifetime of about 2 milliseconds, avoids the use of large distances. This method results in much higher intensities than the usual modulation arrangement. The results show a continuous distribution of neutrons with a rather wide maximum at about 0.8 MV falling off to half of its maximum value at 2.0 MV. The total number of netrons is determined by comparison with the number of fission fragments. The result seems to indicate that only about 30% of the neutrons have energies below .8 MV. Various tests are described which were performed in order to rule out modification of the spectrum by inelastic scattering. Decl. May 4, 1951

  16. Federal government expands compliance initiatives.

    Science.gov (United States)

    Dugan, J K

    1997-09-01

    In 1995, the Federal government initiated Operation Restore Trust to increase enforcement of fraud and abuse regulations in Medicare and Medicaid programs. With the success of the original initiative, the government is expanding the project to additional states and program areas. The initial scrutiny of home health agencies, nursing homes, hospice care, and durable medical equipment is being expanded to managed care plans and acute care hospitals with an eye toward DRG creep. To manage this increased enforcement activity, healthcare organizations should institute comprehensive corporate compliance programs. Such programs should provide a framework that delineates responsibilities and provides a systematic means to resolve issues in a timely manner.

  17. Flow boiling in expanding microchannels

    CERN Document Server

    Alam, Tamanna

    2017-01-01

    This Brief presents an up to date summary of details of the flow boiling heat transfer, pressure drop and instability characteristics; two phase flow patterns of expanding microchannels. Results obtained from the different expanding microscale geometries are presented for comparison and addition to that, comparison with literatures is also performed. Finally, parametric studies are performed and presented in the brief. The findings from this study could help in understanding the complex microscale flow boiling behavior and aid in the design and implementation of reliable compact heat sinks for practical applications.

  18. Expanding the eukaryotic genetic code

    Energy Technology Data Exchange (ETDEWEB)

    Chin, Jason W.; Cropp, T. Ashton; Anderson, J. Christopher; Schultz, Peter G.

    2017-02-28

    This invention provides compositions and methods for producing translational components that expand the number of genetically encoded amino acids in eukaryotic cells. The components include orthogonal tRNAs, orthogonal aminoacyl-tRNA synthetases, orthogonal pairs of tRNAs/synthetases and unnatural amino acids. Proteins and methods of producing proteins with unnatural amino acids in eukaryotic cells are also provided.

  19. ERBU, Expanding Rubber Band Universe

    CERN Document Server

    Soares, Domingos

    2015-01-01

    I put forward a simple unidimensional mechanical analogue of the three-dimensional universe models of modern relativistic cosmology. The main goal of the proposal is the appropriate appreciation of the intrinsic relationship between Hubble's law and the homogeneity of expanding relativistic models.

  20. Expanding the eukaryotic genetic code

    Science.gov (United States)

    Chin, Jason W.; Cropp, T. Ashton; Anderson, J. Christopher; Schultz, Peter G.

    2013-01-22

    This invention provides compositions and methods for producing translational components that expand the number of genetically encoded amino acids in eukaryotic cells. The components include orthogonal tRNAs, orthogonal aminoacyl-tRNA synthetases, orthogonal pairs of tRNAs/synthetases and unnatural amino acids. Proteins and methods of producing proteins with unnatural amino acids in eukaryotic cells are also provided.

  1. Expandable Shelter/Container Report.

    Science.gov (United States)

    1973-06-01

    without removing whatever payload might be in the contai ner. Equ i pment located in the expanded porti on of the ES/C durin g norma l operat i ons is...and Supply BattalIon , Div isi on Support Coianand. In addition , divisional avIation battalions have an A Irc raft Maintenance Company. The TOE

  2. Spectrum Trading for Efficient Spectrum Utilization

    Directory of Open Access Journals (Sweden)

    Cong Xiong

    2014-04-01

    Full Text Available The conventional command and control based spectrum management has led to substantial underutilization of some spectrum bands while severely crowding others due to the uneven and dynamic needs that vary over time and at different locations. Spectrum trading has emerged as a promising management approach to substantially improve spectrum utilization and user experience in wireless communications by taking advantage of market-based mechanisms. This article presents an overview of spectrum trading, including the fundamental characteristics of spectrum trading markets, the state-of-the-art techniques for modeling and resolving various spectrum trading issues, and trading based dynamic spectrum sharing and access. Moreover, some open issues in spectrum trading are identified for future research in this area.

  3. 47 CFR 64.1401 - Expanded interconnection.

    Science.gov (United States)

    2010-10-01

    ... 47 Telecommunication 3 2010-10-01 2010-10-01 false Expanded interconnection. 64.1401 Section 64...) MISCELLANEOUS RULES RELATING TO COMMON CARRIERS Expanded Interconnection § 64.1401 Expanded interconnection. (a... 69, subpart G of this chapter, shall offer expanded interconnection for interstate special...

  4. SPECTRUM OF OPPORTUNISTIC INFECTIONS IN HIV-AIDS PATIENTS

    OpenAIRE

    S S Madkar; Ashok Jaykumar Vankudre; SL Nilekar

    2012-01-01

    Aim: AIDS is characterized by a number of opportunistic infections which are responsible for high morbidity and mortality. The spectrum and distribution of opportunistic infections (OIs) in AIDS patients is ever-expanding. This spectrum varies from continent to continent. The aim of the present study was to document the spectrum of OIs in HIV-infected patients in Ambajogai. Material and Method: 178 HIV positive symptomatic patients, either hospitalized or coming to ART (Antiretroviral Therapy...

  5. Expanding the Game Design Space

    DEFF Research Database (Denmark)

    Larsen, Lasse Juel; Majgaard, Gunver

    2016-01-01

    This article considers game design research in educational settings. Its focus is on how undergraduate students – particularly engineering students – learn computer game design. From observations conducted during our game design courses we have developed a model of expanded game design space......, providing a clear game design assignment that involves the formulation of intended player experience and a description of game mechanics. The second layer focuses on game design thinking from six different aspects of game design chosen in relation to the framing of the game design assignment. The third....... It encapsulates the entire development process from the first ideas to the final game with emphasis on game design thinking. Our model of expanded game design space consists of four separate – yet interconnected – layers in the process of game development. The first layer addresses the importance of framing...

  6. Gastrointestinal symptoms and autism spectrum disorder: links and risks – a possible new overlap syndrome

    Directory of Open Access Journals (Sweden)

    Wasilewska J

    2015-09-01

    Full Text Available Jolanta Wasilewska, Mark Klukowski Department of Pediatrics, Gastroenterology and Allergology, Medical University of Bialystok, Bialystok, Poland Abstract: Autism spectrum disorder (ASD is a genetically determined neurodevelopmental brain disorder presenting with restricted, repetitive patterns of behaviors, interests, and activities, or persistent deficits in social communication and social interaction. ASD is characterized by many different clinical endophenotypes and is potentially linked with certain comorbidities. According to current recommendations, children with ASD are at risk of having alimentary tract disorders – mainly, they are at a greater risk of general gastrointestinal (GI concerns, constipation, diarrhea, and abdominal pain. GI symptoms may overlap with ASD core symptoms through different mechanisms. These mechanisms include multilevel pathways in the gut–brain axis contributing to alterations in behavior and cognition. Shared pathogenetic factors and pathophysiological mechanisms possibly linking ASD and GI disturbances, as shown by most recent studies, include intestinal inflammation with or without autoimmunity, immunoglobulin E-mediated and/or cell-mediated GI food allergies as well as gluten-related disorders (celiac disease, wheat allergy, non-celiac gluten sensitivity, visceral hypersensitivity linked with functional abdominal pain, and dysautonomia linked with GI dysmotility and gastroesophageal reflux. Dysregulation of the gut microbiome has also been shown to be involved in modulating GI functions with the ability to affect intestinal permeability, mucosal immune function, and intestinal motility and sensitivity. Metabolic activity of the microbiome and dietary components are currently suspected to be associated with alterations in behavior and cognition also in patients with other neurodegenerative diseases. All the above-listed GI factors may contribute to brain dysfunction and neuroinflammation depending upon

  7. Unconventional microfluidics: expanding the discipline.

    Science.gov (United States)

    Nawaz, Ahmad Ahsan; Mao, Xiaole; Stratton, Zackary S; Huang, Tony Jun

    2013-04-21

    Since its inception, the discipline of microfluidics has been harnessed for innovations in the biomedicine/chemistry fields-and to great effect. This success has had the natural side-effect of stereotyping microfluidics as a platform for medical diagnostics and miniaturized lab processes. But microfluidics has more to offer. And very recently, some researchers have successfully applied microfluidics to fields outside its traditional domains. In this Focus article, we highlight notable examples of such "unconventional" microfluidics applications (e.g., robotics, electronics). It is our hope that these early successes in unconventional microfluidics prompt further creativity, and inspire readers to expand the microfluidics discipline.

  8. Expanding Earth and Static Universe: Two Papers of 1935

    CERN Document Server

    Kragh, Helge

    2015-01-01

    The German-born astronomer Jacob K. E. Halm (1866-1944) wrote in 1935 two papers on quite different subjects, one an astrophysically based argument for the expanding Earth and the other a no less original attempt to explain the galactic redshifts on the basis of a static universe. Of course, Halm was wrong in both cases. The second of the papers is reproduced in toto and compared to other early attempts to avoid the expansion of the universe by means of "tired light" explanations of the redshifts. Although often referred to in the literature on the expanding Earth, the content of Halm's first paper is not well known. This article also provides a brief account of Halm's life and scientific career, which included important studies of the solar spectrum (the "limb effect") and the first version of the mass-luminosity relation for stars.

  9. Collapsible truss structure is automatically expandable

    Science.gov (United States)

    1965-01-01

    Coil springs wound with maximum initial tension in a three-truss, closed loop structure form a collapsible truss structure. The truss automatically expands and provides excellent rigidity and close dimensional tolerance when expanded.

  10. Black holes in an expanding universe.

    Science.gov (United States)

    Gibbons, Gary W; Maeda, Kei-ichi

    2010-04-02

    An exact solution representing black holes in an expanding universe is found. The black holes are maximally charged and the universe is expanding with arbitrary equation of state (P = w rho with -1 black hole temperature.

  11. Shear Acceleration in Expanding Flows

    CERN Document Server

    Rieger, F M

    2016-01-01

    Shear flows are naturally expected to occur in astrophysical environments and potential sites of continuous non-thermal Fermi-type particle acceleration. Here we investigate the efficiency of expanding relativistic outflows to facilitate the acceleration of energetic charged particles to higher energies. To this end, the gradual shear acceleration coefficient is derived based on an analytical treatment. The results are applied to the context of the relativistic jets of active galactic nuclei. The inferred acceleration timescale is investigated for a variety of conical flow profiles (i.e., power law, Gaussian, Fermi-Dirac) and compared to the relevant radiative and non-radiative loss timescales. The results exemplify that relativistic shear flows are capable of boosting cosmic-rays to extreme energies. Efficient electron acceleration, on the other hand, requires weak magnetic fields and may thus be accompanied by a delayed onset of particle energization and affect the overall jet appearance (e.g., core, ridge ...

  12. OCT Expanded Clinical Data Analysis

    Science.gov (United States)

    Van Baalen, Mary; Tafreshi, Ali; Patel, Nimesh; Young, Millennia; Mason, Sara; Otto, Christian; Samuels, Brian; Koslovsky, Matthew; Schaefer, Caroline; Taiym, Wafa; hide

    2017-01-01

    Vision changes identified in long duration space fliers has led to a more comprehensive clinical monitoring protocol. Optical Coherence Tomography (OCT) was recently implemented on board the International Space Station in 2013. NASA is collaborating with Heidelberg Engineering to expand our current OCT data analysis capability by implementing a volumetric approach. Volumetric maps will be created by combining the circle scan, the disc block scan, and the radial scan. This assessment may provide additional information about the optic nerve and further characterize changes related microgravity exposure. We will discuss challenges with collection and analysis of OCT data, present the results of this reanalysis and outline the potential benefits and limitations of the additional data.

  13. 整形病区扩张器置入部位感染病原菌分析与感染的临床治疗%Analysis of the bacteria spectrum and clinical treatment of topical skin infection during soft tissue expander implantation

    Institute of Scientific and Technical Information of China (English)

    常宏; 周毕峰; 崔鑫; 苏映军; 马显杰

    2016-01-01

    Objective To analyze the identification and antibiotics susceptibility of infectious bacteria obtained from the topic skin infection during soft tissue expander implantation.Methods A retrospective analysis of 121 cases with local infections during soft tissue expander implantation between 2003 to 2012 in Department of Plastic and Reconstructive Surgery,Xijing Hospital was performed.The efficacy of clinical management of local infection was evaluated.Laboratory examination on exudates bacterial pathogen and antibiotic susceptibility were performed.Results Based on the severity of wound infection,change of dressings,wound debridement,exudates drainage and intravenous antibiotics administration were applied.All infections were successfully controlled although the expanders were removed in 16 cases with severe infections.Total success rate of expansion in this cohort was 89.33%.Twenty-nine exudates samples collected from either skin rupture wounds or skin outlets of connector tubes of externally placed infusion ports in 29 patients underwent bacterial culture and antibiotic susceptibility test.The 23 samples showed positive while the rest 6 samples showed negative on bacterial identification in the bacterial culture test.Staphylococcus aureus strain was found in 20 samples (86.96%),Staphylococcus epidermidis in 1 sample,Klinefelter Citrobacter in 1 sample,and Aeromonas hydrophila in 1 sample.More than 80% of the pathogens were susceptive to cefazolin,oxacillin,and levofloxacin.Conclusions Staphylococcus aureus is the main pathogen of infection at expander implantation site.The local dressing change,surgical debridement,continuous infusion drainage,and the use intravenous antibiotics are the effective managements.%目的 通过对感染病原菌、药敏结果、感染后处理方式及结果的分析总结,为临床工作提供参考.方法 2003年1月至2012年12月在西京整形医院行扩张器置入术后感染患者共121例,统计分析感染病原菌

  14. Expanding mTOR signaling

    Institute of Scientific and Technical Information of China (English)

    Qian Yang; Kun-Liang Guan

    2007-01-01

    The mammalian target of rapamycin (mTOR) has drawn much attention recently because of its essential role in cell growth control and its involvement in human tumorigenesis. Great endeavors have been made to elucidate the functions and regulation of mTOR in the past decade. The current prevailing view is that mTOR regulates many fundamental biological processes, such as cell growth and survival, by integrating both intracellular and extracellular signals, including growth factors, nutrients, energy levels, and cellular stress. The significance of mTOR has been highlighted most recently by the identification of mTOR-associated proteins. Amazingly, when bound to different proteins, mTOR forms distinctive complexes with very different physiological functions. These findings not only expand the roles that mTOR plays in cells but also further complicate the regulation network. Thus, it is now even more critical that we precisely understand the underlying molecular mechanisms in order to directly guide the development and usage of anti-cancer drugs targeting the mTOR signaling pathway. In this review, we will discuss different mTOR-associated proteins, the regulation of mTOR complexes, and the consequences of mTOR dysregulation under pathophysiological conditions.

  15. Expanding discourse repertoires with hybridity

    Science.gov (United States)

    Kelly, Gregory J.

    2012-09-01

    In "Hybrid discourse practice and science learning" Kamberelis and Wehunt present a theoretically rich argument about the potential of hybrid discourses for science learning. These discourses draw from different forms of "talk, social practice, and material practices" to create interactions that are "intertextually complex" and "interactionally dynamic." The hybrid discourse practices are described as involving the dynamic interplay of at least three key elements: "the lamination of multiple cultural frames, the shifting relations between people and their discourse, and the shifting power relations between and among people." Each of these elements requires a respective unit of analysis and are often mutually reinforcing. The authors present a theoretically cogent argument for the study of hybrid discourse practices and identify the potential such discourses may have for science education. This theoretical development leads to an analysis of spoken and written discourse around a set of educational events concerning the investigation of owl pellets by two fifth grade students, their classmates, and teacher. Two discourse segments are presented and analyzed by the authors in detail. The first is a discourse analysis of the dissection of the owl pellet by two students, Kyle and Max. The second analysis examines the science report of these same two students. In this article, I pose a number of questions about the study with the hope that by doing so I expand the conversation around the insightful analysis presented.

  16. Discovery of Uniformly Expanding Universe

    Directory of Open Access Journals (Sweden)

    Cahill R. T.

    2012-01-01

    Full Text Available Saul Perlmutter and the Brian Schmidt – Adam Riess teams reported that their Friedmann-model GR-based analysis of their supernovae magnitude-redshift data re- vealed a new phenomenon of “dark energy” which, it is claimed, forms 73% of the energy / matter density of the present-epoch universe, and which is linked to the further claim of an accelerating expansion of the universe. In 2011 Perlmutter, Schmidt and Riess received the Nobel Prize in Physics “for the discovery of the accelerating ex- pansion of the Universe through observations of distant supernovae”. Here it is shown that (i a generic model-independent analysis of this data reveals a uniformly expanding universe, (ii their analysis actually used Newtonian gravity, and finally (iii the data, as well as the CMB fluctuation data, does not require “dark energy” nor “dark matter”, but instead reveals the phenomenon of a dynamical space, which is absent from the Friedmann model.

  17. Expansion of the ARX spectrum.

    Science.gov (United States)

    Wallerstein, Robert; Sugalski, Rachel; Cohn, Leora; Jawetz, Robert; Friez, Michael

    2008-06-01

    We present four patients with ARX mutations and widely variant clinical presentations. Case 1, a female with a known ARX mutation has refractory infantile spasms and severe mental retardation. Case 2, a male presented with a neurodegenerative disorder and has a known ARX mutation likely de novo as mother is not a carrier. Cases 3 and 4, two siblings with a novel variant in ARX, which is not clearly pathogenic, have developmental delay. One of the siblings had a diagnosis of autistic spectrum disorder, failure to thrive with severe feeding difficulties, intracranial hemorrhage, and seizures. There are very few affected females with ARX related infantile spasms. These cases expand the known phenotype of this emerging condition.

  18. Expanding the Interaction Equivalency Theorem

    Directory of Open Access Journals (Sweden)

    Brenda Cecilia Padilla Rodriguez

    2015-06-01

    Full Text Available Although interaction is recognised as a key element for learning, its incorporation in online courses can be challenging. The interaction equivalency theorem provides guidelines: Meaningful learning can be supported as long as one of three types of interactions (learner-content, learner-teacher and learner-learner is present at a high level. This study sought to apply this theorem to the corporate sector, and to expand it to include other indicators of course effectiveness: satisfaction, knowledge transfer, business results and return on expectations. A large Mexican organisation participated in this research, with 146 learners, 30 teachers and 3 academic assistants. Three versions of an online course were designed, each emphasising a different type of interaction. Data were collected through surveys, exams, observations, activity logs, think aloud protocols and sales records. All course versions yielded high levels of effectiveness, in terms of satisfaction, learning and return on expectations. Yet, course design did not dictate the types of interactions in which students engaged within the courses. Findings suggest that the interaction equivalency theorem can be reformulated as follows: In corporate settings, an online course can be effective in terms of satisfaction, learning, knowledge transfer, business results and return on expectations, as long as (a at least one of three types of interaction (learner-content, learner-teacher or learner-learner features prominently in the design of the course, and (b course delivery is consistent with the chosen type of interaction. Focusing on only one type of interaction carries a high risk of confusion, disengagement or missed learning opportunities, which can be managed by incorporating other forms of interactions.

  19. Heuristics to Evaluate Interactive Systems for Children with Autism Spectrum Disorder (ASD): e0132187

    National Research Council Canada - National Science Library

    Kamran Khowaja; Siti Salwah Salim

    2015-01-01

      In this paper, we adapted and expanded a set of guidelines, also known as heuristics, to evaluate the usability of software to now be appropriate for software aimed at children with autism spectrum disorder (ASD...

  20. Forests vanish as population expands.

    Science.gov (United States)

    1992-01-01

    Madagascar's forests have been reduced to a narrowing strip along the eastern escarpment. Of the original forest cover of 11.2 million hectares, only 7.6 million remained in 1950. Today this has been halved to 3.8 million hectares--which means the habitat for the island's unique wildlife has been halved, in just 40 years. Every year some 3% of the remaining forest is cleared, almost all of that to provide land for populations expanding at 3.2% a year. The story of 1 village, Ambodiaviavy, near Ranomafana, shows the process at work. 50 years ago the whole area was dense forest. 8 families, 32 people in all, came here in 1947, after French colonials burned down their old village. At first the new settlers farmed only the valley bottoms, easily irrigated by the stream running down from the hilltops. There was no shortage of land. Each family took as much as they were capable of working. Over the next 43 years, the village population swelled 10 times over, to 320, and the number of families grew to 36. Natural growth was supplemented by immigration from the overcrowded plateau, where all cultivable land was occupied. The valley bottom lands had filled up completely by the 1950s. New couples started to clear forest on the sloping valley sides. They moved gradually uphill until today, they are 2/3 of the way to the hilltops. There was a parallel decline in the size of each family's paddy holding--also fueled by population growth. When children marry, parents have to subdivide their own land and give them a plot. So holding in the irrigated valley bottoms have dwindled. Today only a few are big enough to feed a family. The more children in a family, the smaller their share as adults will be. The village chief lives in a small mud hut, looking out over a valley he once owned entirely. Since then he has had 10 children, and given parcels away to each. Though he is the wealthiest man in the village in cattle, his son are among the poorest. They 1/2 a hectare of paddy each

  1. Time to Grow: Year Two Report on ExpandED Schools. A TASC Report

    Science.gov (United States)

    Traill, Saskia; Brohawn, Katie

    2014-01-01

    An analysis of data from the second year of The After-School Corporation's (TASC's) national demonstration of an expanded school day for elementary and middle school students shows that ExpandED Schools improved school culture, decreased rates of students' chronic absenteeism and helped students develop positive learning habits and attitudes.…

  2. Two-electron Oxidation of a Twisted Non Anti-aromatic 40π Expanded Isophlorin

    Indian Academy of Sciences (India)

    PRACHI GUPTA; SANTOSH P PANCHAL; VENKATARAMANARAO G ANAND

    2016-11-01

    Expanded isophlorins are typical examples for stable anti-aromatic systems. Paratropic ring current effects are observed in their NMR spectra mainly due to their planar conformation. Herein we report the synthesis of the first twisted 40π expanded isophlorin and also its two-electron oxidation to a 38π dication. It sustains the twisted conformation for the 4nπ and (4n+2)π electrons. Due to the non-planar conformation, they do not display ring current effects in their respective 1H NMR spectrum. NICS calculations reveal the non-(anti)aromatic features for the neutral 40π and the 38π dication species.

  3. Small nerve fibres, small hands and small feet: a new syndrome of pain, dysautonomia and acromesomelia in a kindred with a novel NaV1.7 mutation.

    Science.gov (United States)

    Hoeijmakers, Janneke G J; Han, Chongyang; Merkies, Ingemar S J; Macala, Lawrence J; Lauria, Giuseppe; Gerrits, Monique M; Dib-Hajj, Sulayman D; Faber, Catharina G; Waxman, Stephen G

    2012-02-01

    The Na(V)1.7 sodium channel is preferentially expressed within dorsal root ganglion and sympathetic ganglion neurons and their small-diameter peripheral axons. Gain-of-function variants of Na(V)1.7 have recently been described in patients with painful small fibre neuropathy and no other apparent cause. Here, we describe a novel syndrome of pain, dysautonomia, small hands and small feet in a kindred carrying a novel Na(V)1.7 mutation. A 35-year-old male presented with erythema and burning pain in the hands since early childhood, later disseminating to the feet, cheeks and ears. He also experienced progressive muscle cramps, profound sweating, bowel disturbances (diarrhoea or constipation), episodic dry eyes and mouth, hot flashes, and erectile dysfunction. Neurological examination was normal. Physical examination was remarkable in revealing small hands and feet (acromesomelia). Blood examination and nerve conduction studies were unremarkable. Intra-epidermal nerve fibre density was significantly reduced compared to age- and sex-matched normative values. The patient's brother and father reported similar complaints including distal extremity redness and pain, and demonstrated comparable distal limb under-development. Quantitative sensory testing revealed impaired warmth sensation in the proband, father and brother. Genetic analysis revealed a novel missense mutation in the SCN9A gene encoding sodium channel Na(V)1.7 (G856D; c.2567G > A) in all three affected subjects, but not in unaffected family members. Functional analysis demonstrated that the mutation hyperpolarizes (-9.3 mV) channel activation, depolarizes (+6.2 mV) steady-state fast-inactivation, slows deactivation and enhances persistent current and the response to slow ramp stimuli by 10- to 11-fold compared with wild-type Na(V)1.7 channels. Current-clamp analysis of dorsal root ganglion neurons transfected with G856D mutant channels demonstrated depolarized resting potential, reduced current threshold

  4. Nonthermal radiation from relativistic electrons accelerated at spherically expanding shocks

    CERN Document Server

    Kang, Hyesung

    2014-01-01

    We study the evolution of the energy spectrum of cosmic-ray electrons accelerated at spherically expanding shocks with low Mach numbers and the ensuing spectral signatures imprinted in radio synchrotron emission. Time-dependent simulations of diffusive shock acceleration (DSA) of electrons in the test-particle limit have been performed for spherical shocks with the parameters relevant for typical shocks in the intracluster medium. The electron and radiation spectra at the shock location can be described properly by the test-particle DSA predictions with the instantaneous shock parameters. However, the volume integrated spectra of both electrons and radiation deviate significantly from the test-particle power-laws, because the shock compression ratio and the flux of injected electrons at the shock gradually decrease as the shock slows down in time. So one needs to be cautious about interpreting observed radio spectra of evolving shocks by simple DSA models in the test-particle regime.

  5. Brief Measures of Anxiety in Non-Treatment-Seeking Youth with Autism Spectrum Disorder

    Science.gov (United States)

    Kerns, Connor Morrow; Maddox, Brenna B.; Kendall, Philip C.; Rump, Keiran; Berry, Leandra; Schultz, Robert T.; Souders, Margaret C.; Bennett, Amanda; Herrington, John; Miller, Judith

    2015-01-01

    This study investigated the accuracy of brief anxiety scales for non-treatment-seeking youth with autism spectrum disorder. In all, 54 youth (7-17?years; IQ: 67-158) with autism spectrum disorder and their parents completed (a) an expanded version of the Anxiety Disorders Interview Schedule--Child/Parent designed to capture typical and atypical…

  6. Brief Measures of Anxiety in Non-Treatment-Seeking Youth with Autism Spectrum Disorder

    Science.gov (United States)

    Kerns, Connor Morrow; Maddox, Brenna B.; Kendall, Philip C.; Rump, Keiran; Berry, Leandra; Schultz, Robert T.; Souders, Margaret C.; Bennett, Amanda; Herrington, John; Miller, Judith

    2015-01-01

    This study investigated the accuracy of brief anxiety scales for non-treatment-seeking youth with autism spectrum disorder. In all, 54 youth (7-17?years; IQ: 67-158) with autism spectrum disorder and their parents completed (a) an expanded version of the Anxiety Disorders Interview Schedule--Child/Parent designed to capture typical and atypical…

  7. PROLANG: an expandable software in protein chemistry.

    Science.gov (United States)

    Petrilli, P; Caporale, C; Sepe, C

    1990-04-01

    PROLANG is an improved version of the PROSOFT program. Improvements to the old commands were made and new ones were added, PROLANG is an open software that users with BASIC programming experience can easily expand.

  8. Confining caesium in expanded natural Perlite

    OpenAIRE

    Rehspringer, Jean Luc; Balencie, J.; Vilminot, Serge; Burger, Didier; Boos, Anne; Estournès, Claude

    2007-01-01

    International audience; We present the potential use of expanded perlite, a metastable amorphous hydrated aluminum silicate, as a permanent medium for the long-term confinement of caesium. A simple loading by mixing an aqueous caesium nitrate solution and expanded perlite at 300K followed by thermal annealing leads to 96% sintering. The formation of pollucite, CsAlSi2O6, a naturally occurring mineral phase, appears as the crystalline phase embedded in a glassy phase. Leaching tests on the res...

  9. Flexible PVC flame retarded with expandable graphite

    CSIR Research Space (South Africa)

    Focke, WW

    2014-02-01

    Full Text Available by the polymer matrix and the exfoliating graphite prevents the formation of a flammable air fuel mixture. Keywords: Expandable graphite; graphite oxide; graphite intercalation compound; exfoliation; thermal analysis ________________ *Corresponding author: Tel... char residue [6] and this contributes to the mechanisms of flame retardant action [5]. Expandable graphite (EG) is a partially oxidized form of graphite containing intercalated guest species (e.g., sulfuric acid anions) in-between the stacked...

  10. Autism Spectrum Disorder (ASD)

    Science.gov (United States)

    ... Español (Spanish) Recommend on Facebook Tweet Share Compartir Autism spectrum disorder (ASD) is a developmental disability that can cause ... factors that may put children at risk for autism spectrum disorder (ASD) and other developmental disabilities. More E-mail ...

  11. The CMBR spectrum

    Energy Technology Data Exchange (ETDEWEB)

    Stebbins, A.

    1997-05-01

    Here we give an introduction to the observed spectrum of the Cosmic Microwave Background Radiation (CMBR) and discuss what can be learned about it. Particular attention will be given to how Compton scattering can distort the spectrum of the CMBR. An incomplete bibliography of relevant papers is also provided.

  12. 5G Spectrum Sharing

    OpenAIRE

    Nekovee, Maziar; Rudd, Richard

    2017-01-01

    In this paper an overview is given of the current status of 5G industry standards, spectrum allocation and use cases, followed by initial investigations of new opportunities for spectrum sharing in 5G using cognitive radio techniques, considering both licensed and unlicensed scenarios. A particular attention is given to sharing millimeter-wave frequencies, which are of prominent importance for 5G.

  13. Fetal Alcohol Spectrum Disorder

    Science.gov (United States)

    Caley, Linda M.; Kramer, Charlotte; Robinson, Luther K.

    2005-01-01

    Fetal alcohol spectrum disorder (FASD) is a serious and widespread problem in this country. Positioned within the community with links to children, families, and healthcare systems, school nurses are a critical element in the prevention and treatment of those affected by fetal alcohol spectrum disorder. Although most school nurses are familiar…

  14. Planning the milking center in expanding dairies.

    Science.gov (United States)

    Smith, J F; Armstrong, D V; Gamroth, M J; Martin, J G

    1997-08-01

    This paper focuses on dairies that want to expand and milk more cows through an existing parlor or by building new parlor. The expansion process can be divided into the following three phases: 1) financial evaluation, 2) design, and 3) construction. A financial evaluation should be carried out first to determine the resources that are available for expanding the dairy operation. This phase is extremely important, considering that 68% of the dairies that expand have cash flow problems within the first 2 yr of operation. The next phase is to design the milking center, and options include expanding the present parlor or constructing a new parlor. The present parlor can be expanded by addition of stalls, but group size must also expand to maintain cow flow at the larger parlor size. Group size can often be increased by combining corrals or free-stall alleys. The third phase in the expansion is to determine the most efficient milking procedure during the construction or remodeling of the facilities.

  15. Wave Reflection Coefficient Spectrum

    Institute of Scientific and Technical Information of China (English)

    俞聿修; 邵利民; 柳淑学

    2003-01-01

    The wave reflection coefficient frequency spectrum and directional spectrum for concrete face slope breakwaters and rubble mound breakwaters are investigated through physical model tests in the present study. The reflection coefficients of oblique irregular waves are analyzed by the Modified Two-Point Method (MTPM) proposed by the authors. The results show that the wave reflection coefficient decreases with increasing wave frequency and incident angle or decreasing structure slope. The reflection coefficient frequency spectrum and its variation with Iribarren number are given in this paper. The paper also suggests an empirical 3-dimensional reflection coefficient spectrum, i.e. reflection coefficient directional spectrum, which can be used to illustrate quantitatively the variation of reflection coefficient with the incident angle and the Iribarren number for oblique irregular waves.

  16. Cosmic Ray Spectrum in Supernova Remnant Shocks

    Science.gov (United States)

    Kang, H.

    2011-10-01

    We performed kinetic simulations of diffusive shock acceleration in Type Ia supernova remnants (SNRs) expanding into a uniform interstellar medium (ISM). The preshock gas temperature is the primary parameter that governs the cosmic ray (CR) acceleration, while magnetic field strength and CR injection rate are secondary parameters. SNRs in the hot ISM, with an injection fraction smaller than 10-4, are inefficient accelerators with less than 10 % energy getting converted to CRs. The shock structure is almost test-particle like and the ensuing CR spectrum can be steeper than E-2. Although the particles can be accelerated to the knee energy of 1015.5ZeV with amplified magnetic fields in the precursor, Alfvénic drift of scattering centers softens the source spectrum as steep as E-2.1 and reduces the CR acceleration efficiency.

  17. Cosmic Ray Spectrum in Supernova Remnant Shocks

    CERN Document Server

    Kang, Hyesung

    2011-01-01

    We performed kinetic simulations of diffusive shock acceleration in Type Ia supernova remnants (SNRs) expanding into a uniform interstellar medium (ISM). The preshock gas temperature is the primary parameter that governs the cosmic ray (CR) acceleration, while magnetic field strength and CR injection rate are secondary parameters. SNRs in the hot ISM, with an injection fraction smaller than 10^{-4}, are inefficient accelerators with less than 10 % energy getting converted to CRs. The shock structure is almost test-particle like and the ensuing CR spectrum can be steeper than E^{-2}. Although the particles can be accelerated to the knee energy of 10^{15.5}Z eV with amplified magnetic fields in the precursor, Alfv'enic drift of scattering centers softens the source spectrum as steep as E^{-2.1} and reduces the CR acceleration efficiency.

  18. Expanded Schools: Developing Mindsets to Support Academic Success. Research Brief

    Science.gov (United States)

    ExpandED Schools, 2014

    2014-01-01

    The national demonstration of ExpandED Schools, The After-School Corporation's (TASC) expanded learning model, was launched in 2011-12 in New York City, Baltimore, and New Orleans. The ExpandED Schools demonstration is being evaluated by Policy Studies Associates (PSA) and is rolling out at a time when there is heightened awareness among…

  19. Probability-consistent spectrum and code spectrum

    Institute of Scientific and Technical Information of China (English)

    沈建文; 石树中

    2004-01-01

    In the seismic safety evaluation (SSE) for key projects, the probability-consistent spectrum (PCS), usually obtained from probabilistic seismic hazard analysis (PSHA), is not consistent with the design response spectrum given by Code for Seismic Design of Buildings (GB50011-2001). Sometimes, there may be a remarkable difference between them. If the PCS is lower than the corresponding code design response spectrum (CDS), the seismic fortification criterion for the key projects would be lower than that for the general industry and civil buildings. In the paper, the relation between PCS and CDS is discussed by using the ideal simple potential seismic source. The results show that in the most areas influenced mainly by the potential sources of the epicentral earthquakes and the regional earthquakes, PCS is generally lower than CDS in the long periods. We point out that the long-period response spectra of the code should be further studied and combined with the probability method of seismic zoning as much as possible. Because of the uncertainties in SSE, it should be prudent to use the long-period response spectra given by SSE for key projects when they are lower than CDS.

  20. Cosmic Ray Spectrum in Supernova Remnant Shocks

    CERN Document Server

    Kang, Hyesung

    2010-01-01

    We perform kinetic simulations of diffusive shock acceleration (DSA) in Type Ia supernova remnants (SNRs) expanding into a uniform interstellar medium (ISM). Bohm-like diffusion assumed, and simple models for Alfvenic drift and dissipation are adopted. Phenomenological models for thermal leakage injection are considered as well. We find that the preshock gas temperature is the primary parameter that governs the cosmic ray (CR) acceleration efficiency and energy spectrum, while the CR injection rate is a secondary parameter. For SNRs in the warm ISM, if the injection fraction is larger than 10^{-4}, the DSA is efficient enough to convert more than 20 % of the SN explosion energy into CRs and the accelerated CR spectrum exhibits a concave curvature flattening to E^{-1.6}. Such a flat source spectrum near the knee energy, however, may not be reconciled with the CR spectrum observed at Earth. On the other hand, SNRs in the hot ISM, with an injection fraction smaller than 10^{-4}, are inefficient accelerators with...

  1. CMB Anisotropy due to Cosmic Strings in an Accelerated Expanding Universe

    CERN Document Server

    Rokni, S Y; Bordbar, M R

    2013-01-01

    We want to find the cosmological constant influence on cosmic microwave background (CMB) anisotropy due to cosmic strings. Considering the space-time metric of a cosmic string under the effect of a positive cosmological constant, the CMB anisotropy is studied. The result shows that a positive cosmological constant (i.e. the presence of cosmic strings in an accelerated expanding universe) weakens the anisotropy so that more strong resolution is needed to detect the corresponding influences on the CMB power spectrum.

  2. The marine diversity spectrum

    DEFF Research Database (Denmark)

    Reuman, Daniel C.; Gislason, Henrik; Barnes, Carolyn

    2014-01-01

    of taxonomy (all the species in a region regardless of clade) are much less studied but are equally important and will illuminate a different set of ecological and evolutionary processes. We develop and test a mechanistic model of how diversity varies with body mass in marine ecosystems. The model predicts...... the form of the diversity spectrum', which quantifies the distribution of species' asymptotic body masses, is a species analogue of the classic size spectrum of individuals, and which we have found to be a new and widely applicable description of diversity patterns. The marine diversity spectrum...... is predicted to be approximately linear across an asymptotic mass range spanning seven orders of magnitude. Slope -0 center dot 5 is predicted for the global marine diversity spectrum for all combined pelagic zones of continental shelf seas, and slopes for large regions are predicted to lie between -0 center...

  3. Fast Spectrum Reactors

    CERN Document Server

    Todd, Donald; Tsvetkov, Pavel

    2012-01-01

    Fast Spectrum Reactors presents a detailed overview of world-wide technology contributing to the development of fast spectrum reactors. With a unique focus on the capabilities of fast spectrum reactors to address nuclear waste transmutation issues, in addition to the well-known capabilities of breeding new fuel, this volume describes how fast spectrum reactors contribute to the wide application of nuclear power systems to serve the global nuclear renaissance while minimizing nuclear proliferation concerns. Readers will find an introduction to the sustainable development of nuclear energy and the role of fast reactors, in addition to an economic analysis of nuclear reactors. A section devoted to neutronics offers the current trends in nuclear design, such as performance parameters and the optimization of advanced power systems. The latest findings on fuel management, partitioning and transmutation include the physics, efficiency and strategies of transmutation, homogeneous and heterogeneous recycling, in addit...

  4. Analog model for an expanding universe

    CERN Document Server

    Weinfurtner, S E C

    2004-01-01

    Over the last few years numerous papers concerning analog models for gravity have been published. It was shown that the dynamical equation of several systems (e.g. Bose-Einstein condensates with a sink or a vortex) have the same wave equation as light in a curved-space (e.g. black holes). In the last few months several papers were released which deal with simulations of the universe. In this article the de-Sitter universe will be compared with a freely expanding three-dimensional spherical Bose-Einstein condensate. Initially the condensate is in a harmonic trap, which suddenly will be switched off. At the same time a small perturbation will be injected in the center of the condensate cloud. The motion of the perturbation in the expanding condensate will be discussed, and after some transformations the similarity to an expanding universe will be shown.

  5. Bank Directors’ Perceptions of Expanded Auditor's Reports

    DEFF Research Database (Denmark)

    Boolaky, Pran Krishansing; Quick, Reiner

    2016-01-01

    of expanded audit reports, namely information on the assurance level, materiality levels and key audit matters (KAM), on bank director perceptions of the quality of the financial statements, the audit and the audit report, as well as on their credit approval decisions. We conduct an experiment involving......Subsequent to the financial crisis, standard setters developed suggestions for enhancing the audit function, in order to increase financial stability. One related idea is to expand the audit report disclosed to the public, to ensure that it is fit for purpose. This study investigates the impact...... a sample of 105 bank directors and use ANCOVA to determine the predictors of bank director perceptions and decisions. Our findings suggest that disclosing the assurance level has a significantly positive impact. In contrast, we cannot demonstrate a material effect of expanding the audit report to include...

  6. Spectrum and network measurements

    CERN Document Server

    Witte, Robert A

    2014-01-01

    This new edition of Spectrum and Network Measurements enables readers to understand the basic theory, relate it to measured results, and apply it when creating new designs. This comprehensive treatment of frequency domain measurements successfully consolidates all the pertinent theory into one text. It covers the theory and practice of spectrum and network measurements in electronic systems. It also provides thorough coverage of Fourier analysis, transmission lines, intermodulation distortion, signal-to-noise ratio and S-parameters.

  7. Glucose transporter-1 deficiency syndrome: The expanding clinical and genetic spectrum of a treatable disorder

    NARCIS (Netherlands)

    W.G. Leen (Wilhelmina); J. Klepper (Joerg); M.M. Verbeek (Marcel); M. Leferink (Maike); T. Hofste (Tom); B.G.M. van Engelen (Baziel); R.A. Wevers (Ron); T. Arthur (Todd); N. Bahi-Buisson (Nadia); D. Ballhausen (Diana); J. Bekhof (Jolita); P. van Bogaert (Patrick); I. Carrilho (Inês); B. Chabrol (Brigitte); M.P. Champion (Michael); J. Coldwell (James); P. Clayton (Peter); E. Donner (Elizabeth); A. Evangeliou (Athanasios); F. Ebinger (Friedrich); K. Farrell (Kevin); R.J. Forsyth (Rob); C.G.E.L. de Goede (Christian); S. Gross (Stephanie); S. Grünewald (Sonja); H. Holthausen (Hans); S. Jayawant (Sandeep); K. Lachlan (Katherine); V. Laugel (Vincent); K. Leppig (Kathy); M.J. Lim (Ming); G.M.S. Mancini (Grazia); A.D. Marina; L. Martorell (Loreto); J. McMenamin (Joe); M.E.C. Meuwissen (Marije); H. Mundy (Helen); N.O. Nilsson (Nils); A. Panzer (Axel); B.T. Poll-The; C. Rauscher (Christian); C.M.R. Rouselle (Christophe); I. Sandvig (Inger); T. Scheffner (Thomas); E. Sheridan (Eamonn); N. Simpson (Neil); P. Sykora (Parol); R. Tomlinson (Richard); J. Trounce (John); D.W.M. Webb (David); B. Weschke (Bernhard); H. Scheffer (Hans); M.A. Willemsen (Michél)

    2010-01-01

    textabstractGlucose transporter-1 deficiency syndrome is caused by mutations in the SLC2A1 gene in the majority of patients and results in impaired glucose transport into the brain. From 2004-2008, 132 requests for mutational analysis of the SLC2A1 gene were studied by automated Sanger sequencing an

  8. Expanding the spectrum of HEXA mutations in Indian patients with Tay–Sachs disease

    Directory of Open Access Journals (Sweden)

    Jayesh Sheth

    2014-01-01

    Full Text Available Tay–Sachs disease is an autosomal recessive neurodegenerative disorder occurring due to impaired activity of β-hexosaminidase-A (EC 3.2.1.52, resulting from the mutation in HEXA gene. Very little is known about the molecular pathology of TSD in Indian children except for a few mutations identified by us. The present study is aimed to determine additional mutations leading to Tay–Sachs disease in nine patients confirmed by the deficiency of β-hexosaminidase-A (C (D175A and c.805G>C (p.G269R in one case; and one small 1 bp deletion c.426delT (p.F142LfsX57 and one splice site mutation c.459+4A>C in the other two cases respectively. None of these mutations were detected in 100 chromosomes from healthy individuals of the same ethnic group. Three previously reported missense mutations, (i c.532C>T (p.R178C, (ii c.964G>T (p.D322Y, and (iii c.1385A>T (p.E462V; two nonsense mutations (i c.709C>T (p.Q237X and (ii c.1528C>T (p.R510X, one 4 bp insertion c.1277_1278insTATC (p.Y427IfsX5 and one splice site mutation c.459+5G>A were also identified in six cases. We observe from this study that novel mutations are more frequently observed in Indian patients with Tay–Sachs disease with clustering of ~73% of disease causing mutations in exons 5 to 12. This database can be used for a carrier rate screening in the larger population of the country.

  9. Educational paper: The expanding clinical and immunological spectrum of severe combined immunodeficiency

    NARCIS (Netherlands)

    M. van der Burg (Mirjam); A.R. Gennery (Andy R.)

    2011-01-01

    textabstractSevere combined immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency characterized by absence of functional T lymphocytes. It is a paediatric emergency, which is life-threatening when recognized too late. The clinical presentation varies from the classical

  10. 77 FR 69933 - Expanding the Economic and Innovation Opportunities of Spectrum Through Incentive Auctions

    Science.gov (United States)

    2012-11-21

    ... analogous to the process of packing boxes into a trunk when these boxes have different sizes and values. 15... programming ``algorithm'' (a mathematical recipe for solving a problem). The second uses a simpler mathematical recipe that the Incentive Auction NPRM refers to as a ``sequential'' algorithm. Each involves the...

  11. Expanding the Therapeutic Spectrum of Artemisinin: Activity Against Infectious Diseases Beyond Malaria and Novel Pharmaceutical Developments

    OpenAIRE

    Thomas Efferth; Romero, Marta R.; Anna Rita Bilia; Ahmed Galal Osman; Mahmoud ElSohly; Michael Wink; Rudolf Bauer; Ikhlas Khan; Maria Camilla Bergonzi; Marin, Jose J. G.

    2016-01-01

    The interest of Western medicine in Traditional Chinese Medicine (TCM) as a source of drug leads/new drugs to treat diseases without available efficient therapies has been dramatically augmented in the last decades by the extensive work and the outstanding findings achieved within this kind of medicine. The practice of TCM over thousands of years has equipped scientists with substantial experience with hundreds of plants that led to the discovery of artemisinin (qinghaosu), which is extracted...

  12. The expanding spectrum of HCV-related cryoglobulinemic vasculitis: a narrative review.

    Science.gov (United States)

    Dammacco, Franco; Racanelli, Vito; Russi, Sabino; Sansonno, Domenico

    2016-08-01

    Cryoglobulinemic vasculitis (CV) is a small-to-medium-vessel vasculitis that appears in 10-15 % of patients chronically infected with hepatitis C virus (HCV). The classic symptom triad of CV, purpura/asthenia/arthralgia, is accompanied by clinical features that include glomerulonephritis, neuropathy, interstitial pneumonitis, and cardiomyopathy, ranging in their severity from mild to life threatening. The risk of developing non-Hodgkin lymphoma is also higher. The cumulative 10-year survival rate of CV patients is significantly lower than in the age- and sex-matched general population, with death typically caused by nephropathy, malignancies, liver involvement, and severe infections. Unfailing serological stigmata include both a cryoglobulin IgM fraction with rheumatoid factor activity and decreased complement C4 levels. On peripheral B cells, the expression of the CD81 B cell receptor is reduced while that of the CD19 receptor is increased. A monoclonal B cell lymphocytosis develops in almost one-third of patients. HCV-related proteins (but not HCV-RNA genomic sequences) can be detected on biopsy samples by immunofluorescence and immunohistochemistry and involve the vessel lumen, vessel walls, and the perivascular spaces of the skin, kidney, and peripheral nerves, supporting the pathogenetic role of HCV in the onset of a widespread microvasculitis. Based on the demonstration of HCV infection in the large majority of CV patients, a therapeutic regimen consisting of once-weekly pegylated interferon-α and the daily administration of ribavirin results in a sustained virologic response in ~50 % of patients. In those with refractory and relapsing disease, addition of the anti-CD20 monoclonal antibody rituximab has significantly increased the overall response rates. The extension to CV of latest-generation direct-acting antivirals, strikingly successful in non-CV HCV-positive patients, has yielded high complete response rates according to the few studies published thus far.

  13. Glucose transporter-1 deficiency syndrome : the expanding clinical and genetic spectrum of a treatable disorder

    NARCIS (Netherlands)

    Leen, Wilhelmina G.; Klepper, Joerg; Verbeek, Marcel M.; Leferink, Maike; Hofste, Tom; van Engelen, Baziel G.; Wevers, Ron A.; Arthur, Todd; Bahi-Buisson, Nadia; Ballhausen, Diana; Bekhof, Jolita; van Bogaert, Patrick; Carrilho, Ines; Chabrol, Brigitte; Champion, Michael P.; Coldwell, James; Clayton, Peter; Donner, Elizabeth; Evangeliou, Athanasios; Ebinger, Friedrich; Farrell, Kevin; Forsyth, Rob J.; de Goede, Christian G. E. L.; Gross, Stephanie; Grunewald, Stephanie; Holthausen, Hans; Jayawant, Sandeep; Lachlan, Katherine; Laugel, Vincent; Leppig, Kathy; Lim, Ming J.; Mancini, Grazia; Della Marina, Adela; Martorell, Loreto; McMenamin, Joe; Meuwissen, Marije E. C.; Mundy, Helen; Nilsson, Nils O.; Panzer, Axel; Poll-The, Bwee T.; Rauscher, Christian; Rouselle, Christophe M. R.; Sandvig, Inger; Scheffner, Thomas; Sheridan, Eamonn; Simpson, Neil; Sykora, Parol; Tomlinson, Richard; Trounce, John; Webb, David; Weschke, Bernhard; Scheffer, Hans; Willemsen, Michel A.

    2010-01-01

    Glucose transporter-1 deficiency syndrome is caused by mutations in the SLC2A1 gene in the majority of patients and results in impaired glucose transport into the brain. From 2004-2008, 132 requests for mutational analysis of the SLC2A1 gene were studied by automated Sanger sequencing and multiplex

  14. Expanding the spectrum of HEXA mutations in Indian patients with Tay-Sachs disease.

    Science.gov (United States)

    Sheth, Jayesh; Mistri, Mehul; Datar, Chaitanya; Kalane, Umesh; Patil, Shekhar; Kamate, Mahesh; Shah, Harshuti; Nampoothiri, Sheela; Gupta, Sarita; Sheth, Frenny

    2014-01-01

    Tay-Sachs disease is an autosomal recessive neurodegenerative disorder occurring due to impaired activity of β-hexosaminidase-A (EC 3.2.1.52), resulting from the mutation in HEXA gene. Very little is known about the molecular pathology of TSD in Indian children except for a few mutations identified by us. The present study is aimed to determine additional mutations leading to Tay-Sachs disease in nine patients confirmed by the deficiency of β-hexosaminidase-A (C (D175A) and c.805G>C (p.G269R) in one case; and one small 1 bp deletion c.426delT (p.F142LfsX57) and one splice site mutation c.459+4A>C in the other two cases respectively. None of these mutations were detected in 100 chromosomes from healthy individuals of the same ethnic group. Three previously reported missense mutations, (i) c.532C>T (p.R178C), (ii) c.964G>T (p.D322Y), and (iii) c.1385A>T (p.E462V); two nonsense mutations (i) c.709C>T (p.Q237X) and (ii) c.1528C>T (p.R510X), one 4 bp insertion c.1277_1278insTATC (p.Y427IfsX5) and one splice site mutation c.459+5G>A were also identified in six cases. We observe from this study that novel mutations are more frequently observed in Indian patients with Tay-Sachs disease with clustering of ~ 73% of disease causing mutations in exons 5 to 12. This database can be used for a carrier rate screening in the larger population of the country.

  15. Isolated posterior uveal effusion: expanding the spectrum of the uveal effusion syndrome

    Directory of Open Access Journals (Sweden)

    Pautler SE

    2014-12-01

    Full Text Available Scott E Pautler,1 David J Browning2 1Department of Ophthalmology, University of South Florida, Morsani College of Medicine, Tampa, FL, USA; 2Charlotte Ear Eye Nose and Throat Associates, Charlotte, NC, USA Abstract: Uveal effusion syndrome usually causes peripheral chorioretinal detachment, but posterior effusion may present as isolated macular edema with serous macular detachment in the setting of hyperopia and a thickened posterior choroid. Carbonic anhydrase inhibitors may be effective to treat this condition. Keywords: uveal effusion, serous, macular detachment, macular edema

  16. Expanding the phenotypic spectrum of Lenz-Majewski syndrome: facial palsy, cleft palate and hydrocephalus.

    Science.gov (United States)

    Wattanasirichaigoon, Duangrurdee; Visudtibhan, Anannit; Jaovisidha, Suphaneewan; Laothamatas, Jiraporn; Chunharas, Amornsri

    2004-07-01

    We report a sporadic case of Lenz-Majewski syndrome (LMS) with newly recognized manifestations including facial palsy, cleft palate and hydrocephalus developing later in infancy. The clinical course of the patient and neuroimaging studies are described. Increased intracranial pressure was recognized and treated early with the aim of preventing neurological morbidity.

  17. C9orf72-related disorders: expanding the clinical and genetic spectrum of neurodegenerative diseases

    Directory of Open Access Journals (Sweden)

    Paulo Victor Sgobbi de Souza

    2015-03-01

    Full Text Available Neurodegenerative diseases represent a heterogeneous group of neurological conditions primarily involving dementia, motor neuron disease and movement disorders. They are mostly related to different pathophysiological processes, notably in family forms in which the clinical and genetic heterogeneity are lush. In the last decade, much knowledge has been acumulated about the genetics of neurodegenerative diseases, making it essential in cases of motor neuron disease and frontotemporal dementia the repeat expansions of C9orf72 gene. This review analyzes the main clinical, radiological and genetic aspects of the phenotypes related to the hexanucleotide repeat expansions (GGGGCC of C9orf72 gene. Future studies will aim to further characterize the neuropsychological, imaging and pathological aspects of the extra-motor features of motor neuron disease, and will help to provide a new classification system that is both clinically and biologically relevant.

  18. Expanding the phenotypic spectrum of PORCN variants in two males with syndromic microphthalmia.

    Science.gov (United States)

    Brady, Paul D; Van Esch, Hilde; Fieremans, Nathalie; Froyen, Guy; Slavotinek, Anne; Deprest, Jan; Devriendt, Koenraad; Vermeesch, Joris R

    2015-04-01

    Variants in PORCN are a cause of Goltz-Gorlin syndrome or Focal Dermal Hypoplasia, an X-linked dominant disorder affecting heterozygous females and until now considered to be embryonic lethal in males. Exome sequencing was performed in a family in which two male siblings were characterized by microphthalmia and additional congenital anomalies including diaphragmatic hernia, spina bifida and cardiac defects. Surprisingly, we identified a maternally inherited variant in PORCN present in both males as well as in two female siblings. This represents the first finding of a PORCN variant in non-mosaic males affected with Goltz-Gorlin syndrome. The apparently asymptomatic mother showed extreme skewing of X-inactivation (90%), an asymptomatic female sibling showed skewing of 88%, and the second female sibling affected with cutis aplasia of the scalp showed X-inactivation considered within the normal range.

  19. Expanded Mutational Spectrum in Cohen Syndrome, Tissue Expression, and Transcript Variants of COH1

    NARCIS (Netherlands)

    Seifert, Wenke; Holder-Espinasse, Muriel; Kuehnisch, Jirko; Kahrizi, Kimia; Tzschach, Andreas; Garshasbi, Masoud; Najmabadi, Hossein; Kuss, Andreas Walter; Kress, Wolfram; Laureys, Genevieve; Loeys, Bart; Brilstra, Eva; Mancini, Grazia M. S.; Dollfus, Helene; Dahan, Karin; Apse, Kira; Hennies, Hans Christian; Horn, Denise

    2009-01-01

    Cohen syndrome is characterised by mental retardation, postnatal microcephaly, facial dysmorphism, pigmentary retinopathy, myopia, and intermittent neutropenia. Mutations in COH1 (VPS13B) have been found in patients with Cohen syndrome from diverse ethnic origins. We have carried out mutation analys

  20. Educational paper. The expanding clinical and immunological spectrum of severe combined immunodeficiency.

    Science.gov (United States)

    van der Burg, Mirjam; Gennery, Andy R

    2011-05-01

    Severe combined immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency characterized by absence of functional T lymphocytes. It is a paediatric emergency, which is life-threatening when recognized too late. The clinical presentation varies from the classical form of SCID through atypical SCID to Omenn syndrome. In addition, there is a considerable immunological variation, which can hamper the diagnosis. In this educational review, we describe the immunopathological background, clinical presentations and diagnostic process of SCID, as well as the therapeutic possibilities.

  1. Expanding the Therapeutic Spectrum of Artemisinin: Activity Against Infectious Diseases Beyond Malaria and Novel Pharmaceutical Developments

    Directory of Open Access Journals (Sweden)

    Thomas Efferth

    2016-08-01

    Full Text Available The interest of Western medicine in Traditional Chinese Medicine (TCM as a source of drug leads/new drugs to treat diseases without available efficient therapies has been dramatically augmented in the last decades by the extensive work and the outstanding findings achieved within this kind of medicine. The practice of TCM over thousands of years has equipped scientists with substantial experience with hundreds of plants that led to the discovery of artemisinin (qinghaosu, which is extracted from the medicinal plant Artemisia annua L. (qinghao. The unexpected success of artemisinin in combating malaria has drawn strong attention from the scientific community towards TCM. Artemisinin was discovered by Youyou Tu in 1972. Since then, several novel pharmacological activities based on the well-known properties of the sesquiterpene lactone structure with the oxepane ring and an endoperoxide bridge have been unravelled. Beyond malaria, artemisinin and its derivatives (artemisinins exert profound activities towards other protozoans (Leishmania, Trypanosoma, amoebas, Neospora caninum, and Eimeria tenella, trematodes (Schistosoma, liver flukes, and viruses (human cytomegalovirus, hepatitis B and C viruses. Less clear is the effect against bacteria and fungi. Based on the promising results of artemisinin and the first generation derivatives (artesunate, artemether, arteether, novel drug development strategies have been pursued. These included the synthesis of acetal- and non-acetal-type artemisinin dimeric molecules as well as developing nanotechnological approaches, e.g. artemisinin-based liposomes, niosomes, micelles, solid lipid nanocarriers, nanostructured lipid carriers, nanoparticles, fullerenes and nanotubes. The current review presents an overview on different aspects of artemisinins, including sources, chemistry, biological/pharmacological properties, types of infectious pathogens that are susceptible to artemisinins in vitro and in vivo, in addition to the advancement in their drug delivery systems utilizing pharmaceutical technology. It would be expected that different therapeutic strategies based on the second and third generation artemisinin derivatives and artemisinin-based drug technologies would be available in the near future to treat specific infectious diseases.

  2. Wankel engines as steam expanders: design considerations

    Energy Technology Data Exchange (ETDEWEB)

    Badr, O.; Naik, S.; O' Callaghan, P.W.; Probert, S.D. (Cranfield Inst. of Tech., Bedford (GB). Dept. of Applied Energy)

    1991-01-01

    Rotary Wankel engines offer several advantages compared with turbines and other positive-displacement machines as the expansion devices in low-power-output Rankine-cycle systems. So a Wankel expander was selected as the most appropriate device for a steam Rankine-engine, operating principally as a mini combined heat-and-power unit, providing a mechanical output of 5-20 kW. A computer-aided-design technique for selecting the optimal geometry and location of the ports of the expander is described: the computer programs are available from the authors. Lubrication and possible material combinations are also discussed. (author).

  3. Radiation damping in closed expanding universes

    Science.gov (United States)

    Bernui, Armando

    The dynamics of a coupled model (harmonic oscillator-relativistic scalar field) in Conformal Robertson-Walker (k = +1) spacetimes is investigated. The exact radiation-reaction equation of the source-including the retarded radiation terms due to the closed space geometry - is obtained and analyzed. A suitable family of Lyapunov functions is constructed to show that, if the spacetime expands monotonely, then the source's energy damps. A numerical simulation of this equation for expanding Universes, with and without Future Event Horizon, is performed.

  4. Bound systems in an expanding universe

    CERN Document Server

    Baker, G A

    2000-01-01

    The Schwarzchild solution insertion in an expanding universe, the so-called "Swiss cheese model," is shown to possess an extrinsic curvature miss-match. One consequence is that some trajectories are discontinuous functions of their initial conditions. An alternate metric is proposed which goes smoothly between the Schwarzchild exterior solution and the Friedmann-Lemaitre, expanding universe metric. It is further shown that the effects of the expansion on planetary motions in the solar system are too small to be currently observed for this alternate metric.

  5. Responsible implementation of expanded carrier screening.

    Science.gov (United States)

    Henneman, Lidewij; Borry, Pascal; Chokoshvili, Davit; Cornel, Martina C; van El, Carla G; Forzano, Francesca; Hall, Alison; Howard, Heidi C; Janssens, Sandra; Kayserili, Hülya; Lakeman, Phillis; Lucassen, Anneke; Metcalfe, Sylvia A; Vidmar, Lovro; de Wert, Guido; Dondorp, Wybo J; Peterlin, Borut

    2016-06-01

    This document of the European Society of Human Genetics contains recommendations regarding responsible implementation of expanded carrier screening. Carrier screening is defined here as the detection of carrier status of recessive diseases in couples or persons who do not have an a priori increased risk of being a carrier based on their or their partners' personal or family history. Expanded carrier screening offers carrier screening for multiple autosomal and X-linked recessive disorders, facilitated by new genetic testing technologies, and allows testing of individuals regardless of ancestry or geographic origin. Carrier screening aims to identify couples who have an increased risk of having an affected child in order to facilitate informed reproductive decision making. In previous decades, carrier screening was typically performed for one or few relatively common recessive disorders associated with significant morbidity, reduced life-expectancy and often because of a considerable higher carrier frequency in a specific population for certain diseases. New genetic testing technologies enable the expansion of screening to multiple conditions, genes or sequence variants. Expanded carrier screening panels that have been introduced to date have been advertised and offered to health care professionals and the public on a commercial basis. This document discusses the challenges that expanded carrier screening might pose in the context of the lessons learnt from decades of population-based carrier screening and in the context of existing screening criteria. It aims to contribute to the public and professional discussion and to arrive at better clinical and laboratory practice guidelines.

  6. Expanding the Focus of Career Assessment.

    Science.gov (United States)

    Lock, Jared D.; Hogan, Robert

    2000-01-01

    Issues affecting career assessment include change in the focus and definition of career, emphasis on quality of work life, expansion of career paths, increased amount of career information available on the Internet, and questionable quality of online assessment. An expanded model of career assessment now includes technical fit, personal fit,…

  7. Expanding CTE Opportunities through Blended Learning

    Science.gov (United States)

    McKinstry, Elizabeth

    2012-01-01

    The global economy, 21st century skills, knowledge society, college and career readiness, digital and project-based learning are all common terms to educators who are expanding their learning environments beyond the classroom to meet the needs of all students. It is common knowledge that the rapid technological advances of this century have…

  8. Expanded austenite, crystallography and residual stress

    DEFF Research Database (Denmark)

    Christiansen, Thomas; Hummelshøj, Thomas Strabo; Somers, Marcel A. J.

    2010-01-01

    compositions and (b) unravelling of the contributions of stress-depth and composition-depth profiles in expanded austenite layers are summarised and discussed. It is shown through simulation of line profiles that the combined effects of composition gradients, stress gradients and stacking fault gradients can...

  9. Responsible implementation of expanded carrier screening

    Science.gov (United States)

    Henneman, Lidewij; Borry, Pascal; Chokoshvili, Davit; Cornel, Martina C; van El, Carla G; Forzano, Francesca; Hall, Alison; Howard, Heidi C; Janssens, Sandra; Kayserili, Hülya; Lakeman, Phillis; Lucassen, Anneke; Metcalfe, Sylvia A; Vidmar, Lovro; de Wert, Guido; Dondorp, Wybo J; Peterlin, Borut

    2016-01-01

    This document of the European Society of Human Genetics contains recommendations regarding responsible implementation of expanded carrier screening. Carrier screening is defined here as the detection of carrier status of recessive diseases in couples or persons who do not have an a priori increased risk of being a carrier based on their or their partners' personal or family history. Expanded carrier screening offers carrier screening for multiple autosomal and X-linked recessive disorders, facilitated by new genetic testing technologies, and allows testing of individuals regardless of ancestry or geographic origin. Carrier screening aims to identify couples who have an increased risk of having an affected child in order to facilitate informed reproductive decision making. In previous decades, carrier screening was typically performed for one or few relatively common recessive disorders associated with significant morbidity, reduced life-expectancy and often because of a considerable higher carrier frequency in a specific population for certain diseases. New genetic testing technologies enable the expansion of screening to multiple conditions, genes or sequence variants. Expanded carrier screening panels that have been introduced to date have been advertised and offered to health care professionals and the public on a commercial basis. This document discusses the challenges that expanded carrier screening might pose in the context of the lessons learnt from decades of population-based carrier screening and in the context of existing screening criteria. It aims to contribute to the public and professional discussion and to arrive at better clinical and laboratory practice guidelines. PMID:26980105

  10. Expanding the collaboration between CERN and Pakistan

    CERN Multimedia

    2002-01-01

    Parvez Butt, chairman of the Pakistan Atomic Energy Commission, and CERN Director General, Luciano Maiani, signed a letter of intent last week to expand collaboration. Through an agreement which should be formalized within a few months, Pakistan would make a substantial contribution to the LHC and its detectors, coordinated by the Pakistani National Centre of Physics.

  11. Expanding Educational Excellence: The Power of Schools

    Science.gov (United States)

    Coleman, Mary Ruth; Winn, Donna-Marie; Harradine, Christine

    2012-01-01

    In this paper, the authors explore four major barriers to academic success that must be addressed, briefly describe two projects that have worked to address these barriers, and make recommendations for moving forward as they work to expand educational excellence for all students. They provide examples of the myriad ways in which schools have the…

  12. Expanded austenite; crystallography and residual stress

    DEFF Research Database (Denmark)

    Christiansen, Thomas; Hummelshøj, Thomas Strabo; Somers, Marcel A. J.

    2009-01-01

    The identity of expanded austenite as developing during low temperature nitriding and/or carburizing of austenitic stainless steel has been under debate since the very first observation of this phase. In the present article recent results obtained with i) homogeneous samples of various uniform...

  13. Properties of extruded expandable breadfruit products

    Science.gov (United States)

    Dried breadfruit was extruded with a twin screw extruder to develop a value-added expanded fruit product. This research studied the effects of barrel temperature (120-160°C), moisture content (13-25%), feeding rate (13-25 kg/h) and screw speed (115-175rpm) on physicochemical properties (bulk densit...

  14. Carbohydrate plasma expanders for passive tumor targeting

    DEFF Research Database (Denmark)

    Hoffmann, Stefan; Caysa, Henrike; Kuntsche, Judith

    2013-01-01

    The objective of this study was to investigate the suitability of carbohydrate plasma volume expanders as a novel polymer platform for tumor targeting. Many synthetic polymers have already been synthesized for targeted tumor therapy, but potential advantages of these carbohydrates include...

  15. Expanding Your Horizons Conference in Geneva

    CERN Multimedia

    Chromek-Burckhart, Doris

    2011-01-01

    CERN and its experiments participated in Expanding Your Horizons (EYH) in Science and Mathematics conference in Geneva on 12th November. EYH nurture girls' interest in science and math courses to encourage them to consider careers in science, technology, engineering, and math.

  16. Heat expanded starch-based compositions.

    Science.gov (United States)

    Glenn, Gregory M; Klamczynski, Artur K; Holtman, Kevin M; Shey, Justin; Chiou, Bor-Sen; Berrios, Jose; Wood, Delilah; Orts, William J; Imam, Syed H

    2007-05-16

    A heat expansion process similar to that used for expanded bead polystyrene was used to expand starch-based compositions. Foam beads made by solvent extraction had the appearance of polystyrene beads but did not expand when heated due to an open-cell structure. Nonporous beads, pellets, or particles were made by extrusion or by drying and milling cooked starch slurries. The samples expanded into a low-density foam by heating 190-210 degrees C for more than 20 s at ambient pressures. Formulations containing starch (50-85%), sorbitol (5-15%), glycerol (4-12%), ethylene vinyl alcohol (EVAL, 5-15%), and water (10-20%) were studied. The bulk density was negatively correlated to sorbitol, glycerol, and water content. Increasing the EVAL content increased the bulk density, especially at concentrations higher than 15%. Poly(vinyl alcohol) (PVAL) increased the bulk density more than EVAL. The bulk density was lowest in samples made of wheat and potato starch as compared to corn starch. The expansion temperature for the starch pellets decreased more than 20 degrees C as the moisture content was increased from 10 to 25%. The addition of EVAL in the formulations decreased the equilibrium moisture content of the foam and reduced the water absorption during a 1 h soaking period.

  17. Anaesthesia and familial dysautonomia with congenital insensitivity ...

    African Journals Online (AJOL)

    Adele

    Southern African Journal of Anaesthesia & Analgesia - May 2005. 66. Anaesthesia .... Children with this condition present for a variety of dental, endoscopic, orthopaedic ... A variety of anaesthetic techniques have been described with vary-.

  18. Spondylodiscitis in familial dysautonomia: a case report.

    Science.gov (United States)

    Ghermandi, R; Mesfin, A; Terzi, S; Colangeli, S; Zamparini, E; Gasbarrini, A

    2014-01-01

    Familial dysautomonia (FD, or Riley-Day syndrome) is a rare but fatal autosomal recessive peripheral neuropathy caused by a point mutation in I-κ-B kinase complex associated protein (IKBCAP) gene. The disease, that affects primarily people of Ashkenazi Jewish origin, prejudices the development of primary sensory neurons determining depletion of autonomic and sensory neurons. Musculoskeletal problems include: spinal deformities, foot deformities, fractures and arthopathies. In this article we review a case of a 34 years old male of non-Jewish origin affected by FD presenting L2-L3 kyphosis and inability to walk due to chronic L2-L3 spondylodiscitis not surgically treated 14 years before as acute disease. De novo spondylodiscitis affecting patients presenting FD and its subsequent management was not previously described in the literature.

  19. IR Spectrum Data Bank System

    Institute of Scientific and Technical Information of China (English)

    Xie Dilin

    2004-01-01

    The infrared spectra of pure compounds of ninety thousands, poly compounds of twelve thousand, drugs of one thousand were included in the data bank. All of them can be searched out according to their serial number, chemical name, commercial name, amount of each atoms, or molecular formula, as well as their spectrum peak appearances. Program for spectrum information inputting, program for spectrum information search and program for spectrum peak appearance search were included in the system; in addition, spectrum information data bank, spectrum peak code data bank and spectrum figure data bank were attached to the system. System program was written by Visial Basic, and run under Windows system. The spectrum information data bank and spectrum figure data bank were administrated by Microsoft Access.The program for spectrum message inputting can be used to add message data and spectrum figure of some new compounds into the data banks by users themselves. The program for spectrum message search was designed to find out all the message data and spectrum figure of interested compound according to someone of the message data. The program for spectrum peak search was designed to find out some spectra most similar in peak shape with unknown spectrum by peak to peak comparison. When the wavenumbers and transmittances of main peaks in the spectrum of unknown sample were entered, the spectrum peak search was performed and several hits with higher similarity were reported including their similarity scores, spectrum serial numbers, sample's states,melt points, molecular formulas as well as spectrum images. If the search result was not satisfactory,some methods to modify spectrum parameters were reminded and search was performed again.

  20. Expanding Geometry Understanding with 3D Printing

    Science.gov (United States)

    Cochran, Jill A.; Cochran, Zane; Laney, Kendra; Dean, Mandi

    2016-01-01

    With the rise of personal desktop 3D printing, a wide spectrum of educational opportunities has become available for educators to leverage this technology in their classrooms. Until recently, the ability to create physical 3D models was well beyond the scope, skill, and budget of many schools. However, since desktop 3D printers have become readily…

  1. Expanding Geometry Understanding with 3D Printing

    Science.gov (United States)

    Cochran, Jill A.; Cochran, Zane; Laney, Kendra; Dean, Mandi

    2016-01-01

    With the rise of personal desktop 3D printing, a wide spectrum of educational opportunities has become available for educators to leverage this technology in their classrooms. Until recently, the ability to create physical 3D models was well beyond the scope, skill, and budget of many schools. However, since desktop 3D printers have become readily…

  2. Expanding advanced civilizations in the universe

    CERN Document Server

    Gros, C

    2005-01-01

    The 1950 lunch-table remark by Enrico Fermi `Where is everybody' has started intensive scientific and philosophical discussions about what we call nowadays the `Fermi paradox': If there had been ever a single advanced civilization in the cosmological history of our galaxy, dedicated to expansion, it would have had plenty of time to colonize the entire galaxy via exponential growth. No evidence of present or past alien visits to earth are known to us, leading to the standard conclusion that no advanced expanding civilization has ever existed in the milky-way \\cite{Webb}. This conclusion rest fundamentally on the ad-hoc assumption, that any alien civilizations dedicated to expansion at one time would remain dedicated to expansions forever. Considering our limited knowledge about alien civilizations we need however to relax this basic assumption. Here we show that a substantial and stable population of expanding advanced civilization might consequently exist in our galaxy.

  3. Parameter estimation for an expanding universe

    Directory of Open Access Journals (Sweden)

    Jieci Wang

    2015-03-01

    Full Text Available We study the parameter estimation for excitations of Dirac fields in the expanding Robertson–Walker universe. We employ quantum metrology techniques to demonstrate the possibility for high precision estimation for the volume rate of the expanding universe. We show that the optimal precision of the estimation depends sensitively on the dimensionless mass m˜ and dimensionless momentum k˜ of the Dirac particles. The optimal precision for the ratio estimation peaks at some finite dimensionless mass m˜ and momentum k˜. We find that the precision of the estimation can be improved by choosing the probe state as an eigenvector of the hamiltonian. This occurs because the largest quantum Fisher information is obtained by performing projective measurements implemented by the projectors onto the eigenvectors of specific probe states.

  4. Carbohydrate plasma expanders for passive tumor targeting

    DEFF Research Database (Denmark)

    Hoffmann, Stefan; Caysa, Henrike; Kuntsche, Judith

    2013-01-01

    The objective of this study was to investigate the suitability of carbohydrate plasma volume expanders as a novel polymer platform for tumor targeting. Many synthetic polymers have already been synthesized for targeted tumor therapy, but potential advantages of these carbohydrates include...... inexpensive synthesis, constant availability, a good safety profile, biodegradability and the long clinical use as plasma expanders. Three polymers have been tested for cytotoxicity and cytokine activation in cell cultures and conjugated with a near-infrared fluorescent dye: hydroxyethyl starches (HES 200 k......Da and HES 450 kDa) and dextran (DEX 500 kDa). Particle size and molecular weight distribution were determined by asymmetric flow field-flow fractionation (AF4). The biodistribution was investigated non-invasively in nude mice using multispectral optical imaging. The most promising polymer conjugate...

  5. Hubble, Hubble's law and the expanding universe

    OpenAIRE

    Bagla, J. S.

    2009-01-01

    Hubble's name is associated closely with the idea of an expanding universe as he discovered the relation between the recession velocity and distances of galaxies. Hubble also did a lot of pioneering work on the distribution of galaxies in the universe. In this article we take a look at Hubble's law and discuss how it relates with models of the universe. We also give a historical perspective of the discoveries that led to the Hubble's law.

  6. The Galatia preparation plant expands throughput

    Energy Technology Data Exchange (ETDEWEB)

    Reed, S.R.; Campbell, J.A.L.; Riffey, R.L. [Kerr-McGee Coal, Oklahoma City, OK (United States)

    1995-04-01

    Profiles the Kerr-McGee`s Galatia coal preparation plant, located on the Galatia Mine complex near Marian (Illinois). The plant, first opened in 1984, originally consisted of a coarse heavy-media vessel circuit, dual heavy-media cyclone circuits, and split fine-coal flotation. In 1994 the plant was expanded adding new technology whilst retaining the original design concepts. New technology installed included new spirals and fiberglass replacement components to overcome corrosion. 3 figs.

  7. Inextendibility of expanding cosmological models with symmetry

    Energy Technology Data Exchange (ETDEWEB)

    Dafermos, Mihalis [University of Cambridge, Department of Pure Mathematics and Mathematical Statistics, Wilberforce Road, Cambridge CB3 0WB (United Kingdom); Rendall, Alan D [Max Planck Institute for Gravitational Physics, Albert Einstein Institute, Am Muehlenberg 1, D-14476 Golm (Germany)

    2005-12-07

    A new criterion for inextendibility of expanding cosmological models with symmetry is presented. It is applied to derive a number of new results and to simplify the proofs of existing ones. In particular, it shows that the solutions of the Einstein-Vlasov system with T{sup 2} symmetry, including the vacuum solutions, are inextendible in the future. The technique introduced adds a qualitatively new element to the available tool-kit for studying strong cosmic censorship. (letter to the editor)

  8. Hubble, Hubble's law and the expanding universe

    OpenAIRE

    Bagla, J. S.

    2009-01-01

    Hubble's name is associated closely with the idea of an expanding universe as he discovered the relation between the recession velocity and distances of galaxies. Hubble also did a lot of pioneering work on the distribution of galaxies in the universe. In this article we take a look at Hubble's law and discuss how it relates with models of the universe. We also give a historical perspective of the discoveries that led to the Hubble's law.

  9. GWDC Expands High-End Market Share

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ It is a decision of great significance for GWDC to expand high-end market share in order to realize its transformation of development strategy and improve its development quality. As an important step of GWDC to explore high-end market, Oman PDO Project marks the first time that the Chinese petroleum engineering service team cooperates with the transnational petroleum corporations ranking first three in the world.

  10. Expander Graphs in Pure and Applied Mathematics

    OpenAIRE

    Lubotzky, Alexander

    2011-01-01

    Expander graphs are highly connected sparse finite graphs. They play an important role in computer science as basic building blocks for network constructions, error correcting codes, algorithms and more. In recent years they have started to play an increasing role also in pure mathematics: number theory, group theory, geometry and more. This expository article describes their constructions and various applications in pure and applied mathematics.

  11. Strings, Fivebranes and an Expanding Universe

    OpenAIRE

    2002-01-01

    It was recently shown that velocity-dependent forces between parallel fundamental strings moving apart in a $D-$dimensional spacetime implied an accelerating expanding universe in $D-1$-dimensional space-time. Exact solutions were obtained for the early time expansion in $D=5,6$. Here we show that this result also holds for fundamental strings in the background of a fivebrane, and argue that the feature of an accelerating universe would hold for more general $p$-brane-seeded models.

  12. Cosmic strings in an expanding spacetime

    Energy Technology Data Exchange (ETDEWEB)

    Stein-Schabes, J.A.; Burd, A.B.

    1988-03-15

    We study string solutions in an expanding Friedmann-Robertson-Walker (FRW) spacetime. The back reaction of the string on the spacetime has been ignored so that the background stays Friedmannian throughout the evolution. By numerically integrating the field equations in both radiation- and matter-dominated eras, we discover some new oscillatory solutions. The possible damping of these oscillations is discussed. For late times the solution becomes identical to the static one.

  13. Sinclair ZX Spectrum.

    Science.gov (United States)

    Rodwell, Peter

    1982-01-01

    Describes and evaluates the hardware, software, peripheral devices, performance capabilities, and programing capacity of the Sinclair ZX Spectrum microcomputer. The computer's display system, its version of the BASIC programing language, its graphics capabilities, and the unique features of its data entry keyboard are discussed. (JL)

  14. Autism Spectrum Disorder (ASD)

    Science.gov (United States)

    ... with: 1 Communication and interaction with other people Restricted interests and repetitive behaviors Different people with autism can have different symptoms. For this reason, autism is known as a spectrum disorder —which means that there is a range of ...

  15. Autism Spectrum Disorder

    Centers for Disease Control (CDC) Podcasts

    2014-04-02

    This podcast discusses autism spectrum disorder (ASD), a developmental disability that causes problems with social, communication, and behavioral skills. CDC estimates that one in 68 children has been identified as having ASD.  Created: 4/2/2014 by National Center on Birth Defects and Developmental Disabilities (NCBDDD).   Date Released: 4/2/2014.

  16. What is a Spectrum?

    CERN Document Server

    Bolton, Adam S; Brownstein, Joel; Pandey, Parul; Schlegel, David; Shu, Yiping

    2011-01-01

    This contribution describes the "spectro-perfectionism" algorithm of Bolton & Schlegel (2010, PASP, 122, 248) that is being implemented within the Baryon Oscillation Spectroscopic Survey (BOSS) of the Sloan Digital Sky Survey III (SDSS-III), in terms of its potential to deliver Poisson-limited sky subtraction and lossless compression of the input spectrum likelihood functional given raw CCD data.

  17. Battlefield spectrum management

    Science.gov (United States)

    Sivakumar, C.

    1997-06-01

    Modern tactical communications systems rely on radios to support network and user connectivity. One of the challenges for network planners and managers is to make best use of scarce and vulnerable frequency spectrum resources to support the communication needs of war fighters. With the wide variety of Iris radio types typically to be deployed in the battlefield (ranging from high frequency to super high frequency), a comprehensive suite of tools is necessary to ensure that frequency interference is kept minimum. Without a sophisticated frequency spectrum management system, the most advanced tactical communications systems could be rendered useless, jeopardizing human life and national security. For these reasons, it is important to develop an Iris wide battlefield spectrum management capability that takes full advantage of current frequency spectrum management research and development (R&D), related tools, and supporting technology for assigning frequencies. This session briefly describes various assignment strategies being adopted in the Iris BFSM for overcoming cosite/collocated/farsite interferences along with the propagation models [from high frequency (HF) to super high frequency (SHF)] used for the assignment of frequencies. Also a brief thread outlining the process for generating frequency allocation/assignment request and analysis of frequency interference is discussed.

  18. Inverted porphyrins and expanded porphyrins: An overview

    Indian Academy of Sciences (India)

    S K Pushpan; S Venkatraman; V G Anand; J Sankar; H Rath; T K Chandrashekar

    2002-08-01

    Porphyrins and metallopophyrins have attracted the attention of chemists for the past 100 years or more owing to their widespread involvement in biology. More recently, synthetic porphyrins and porphyrin-like macrocycles have attracted the attention of researchers due to their diverse applications as sensitizers for photodynamic therapy, MRI contrasting agents, and complexing agents for larger metal ions and also for their anion binding abilities. The number of -electrons in the porphyrin ring can be increased either by increasing the number of conjugated double bonds between the pyrrole rings or by increasing the number of heterocyclic rings. Thus, 22 sapphyrins, 26 rubyrins, 30 heptaphyrins, 34 octaphyrins and higher cyclic polypyrrole analogues containing 40, 48, 64, 80 and 96 systems have recently been reported in the literature. These macrocycles show rich structural diversity where normal and different kinds of inverted structures have been identified. In this review, an attempt has been made to collect the literature of the inverted porphyrins and expanded porphyrins reported until December 2001. Since the meso aryl expanded porphyrins have tendency to form both inverted and non-inverted structures more emphasis has been given to meso aryl expanded porphyrins.

  19. Lubricin in human breast tissue expander capsules.

    Science.gov (United States)

    Cheriyan, Thomas; Guo, Lifei; Orgill, Dennis P; Padera, Robert F; Schmid, Thomas M; Spector, Myron

    2012-10-01

    Capsular contraction is the most common complication of breast reconstruction surgery. While presence of the contractile protein alpha smooth muscle actin (α-SMA) is considered among the causes of capsular contraction, the exact etiology and pathophysiology is not fully understood. The objective of this study was to investigate the possible role of lubricin in capsular formation and contraction by determining the presence and distribution of the lubricating protein lubricin in human breast tissue expander capsules. Related aims were to evaluate select histopathologic features of the capsules, and the percentage of cells expressing α-SMA, which reflects the myofibroblast phenotype. Capsules from tissue expanders were obtained from eight patients. Lubricin, at the tissue-implant interface, in the extracellular matrix, and in cells, and α-SMA-containing cells were evaluated immunohistochemically. The notable finding was that lubricin was identified in all tissue expander capsules: as a discrete layer at the tissue-implant interface, extracellular, and intracellular. There was a greater amount of lubricin in the extracellular matrix in the intimal-subintimal zone when compared with the tissue away from the implant. Varying degrees of synovial metaplasia were seen at the tissue-implant interface. α-SMA-containing cells were also seen in all but one patient. The findings might help us better understand factors involved in capsule formation.

  20. Measurements of an expanding surface flashover plasma

    Energy Technology Data Exchange (ETDEWEB)

    Harris, J. R., E-mail: john.harris@colostate.edu [Department of Electrical and Computer Engineering, Colorado State University, Fort Collins, Colorado 80523 (United States)

    2014-05-21

    A better understanding of vacuum surface flashover and the plasma produced by it is of importance for electron and ion sources, as well as advanced accelerators and other vacuum electronic devices. This article describes time-of-flight and biased-probe measurements made on the expanding plasma generated from a vacuum surface flashover discharge. The plasma expanded at velocities of 1.2–6.5 cm/μs, and had typical densities of 10{sup 10}–10{sup 12} cm{sup −3}. The expansion velocity of the plasma leading edge often exhibited a sharp increase at distances of about 50 mm from the discharge site. Comparison with biased-probe data suggests that, under most conditions, the plasma leading edge was dominated by negative ions, with the apparent increase in velocity being due to fast H{sup −} overtaking slower, heavier ions. In some cases, biased-probe data also showed abrupt discontinuities in the plasma energy distribution co-located with large changes in the intercepted plasma current, suggesting the presence of a shock in the leading edge of the expanding plasma.

  1. Optical cavity resonator in an expanding universe

    CERN Document Server

    Kopeikin, Sergei

    2014-01-01

    We study evolution of frequency of a standing electromagnetic (EM) wave in a resonant optical cavity placed to the expanding manifold described by the Robertson-Walker metric. One builds a local coordinate system in which spacetime is locally Minkowskian. However, due to the conformal nature of the Robertson-Walker metric the conventional transformation to the local inertial coordinates introduces ambiguity in the physical interpretation of the local time coordinate. Therefore, contrary to a common-sense expectation, a straightforward implementation of EEP alone does not allow us to decide whether atomic clocks ticks at the same rate as the clocks based on EM modes of a cavity. To resolve the ambiguity we analyzed the cavity rigidity and the oscillation of its EM modes in an expanding universe by employing the Maxwell equations. We found out that both the size of the cavity and the EM frequency experience an adiabatic drift in conformal coordinates as the universe expands. We set up the oscillation equation f...

  2. Distant Supernovae Indicate Ever-Expanding Universe

    Science.gov (United States)

    1998-12-01

    ESO Astronomers Contribute towards Resolution of Cosmic Puzzle Since the discovery of the expansion of the Universe by American astronomer Edwin Hubble in the 1920's, by measurement of galaxy velocities, astronomers have tried to learn how this expansion changes with time. Until now, most scientists have been considering two possibilities: the expansion rate is slowing down and will ultimately either come to a halt - whereafter the Universe would start to contract, or it will continue to expand forever. However, new studies by two independent research teams, based on observations of exploding stars ( supernovae ) by ESO astronomers [1] with astronomical telescopes at the La Silla Observatory as well as those of their colleagues at other institutions, appear to show that the expansion of the Universe is accelerating . The results take the discovery of the cosmological expansion one step further and challenge recent models of the Universe. If the new measurements are indeed correct, they show that the elusive "cosmological constant" , as proposed by Albert Einstein , contributes significantly to the evolution of the Universe. The existence of a non-zero cosmological constant implies that a repulsive force, counter-acting gravity, currently dominates the universal expansion , and consequently leads to an ever-expanding Universe. This new research is being named as the "Breakthrough of the Year" by the renowned US science journal Science in the December 18, 1998, issue. A Press Release is published by the journal on this occasion. "Fundamental Parameters" of the Universe Three fundamental parameters govern all cosmological models based on the theory of General Relativity. They are 1. the current expansion rate as described by Hubble's constant , i.e. the proportionality factor between expansion velocity and distance 2. the average matter density in the Universe, and 3. the amount of "other energy" present in space. From the measured values of these fundamental

  3. From passive to active: The spectrum of peace journalism

    Directory of Open Access Journals (Sweden)

    Jesse Benn

    2015-10-01

    Full Text Available One of the biggest hurdles facing the field of peace journalism (PJ is its vague definition. This paper proposes defining PJ as it operates on a spectrum, from passive to active. Through a review of extant PJ literature, this paper synthesizes current theory into an overarching, explicit concept, and calls for it to be further adopted and expanded. To conclude it considers potential advantages, drawbacks, and critiques of its proposal.

  4. Spectrum Policy in the Age of Broadband: Issues for Congress

    Science.gov (United States)

    2010-01-29

    homes or convenient public locations, including airports and café environments such as Starbucks . In 2008, there were over 7,000 Starbucks locations...of by a license-holder. 135 “ Starbucks to Expand Technology Relationship with AT&T,” Starbucks Corporation Press Room, February 11, 2008, http...technologies and to formulate new investment strategies . The possibility of allocating additional spectrum for mobile broadband was among the

  5. New Aspects of Photon Propagation in Expanding Universes

    CERN Document Server

    Fahr, H -J

    2016-01-01

    According to present cosmological views the energy density of CMB (Cosmic Microwave Background) photons, freely propagating through the expanding cosmos, varies proportional to 1/S^4 with S being the scale factor of the universe. This behavior is expected, because General Theory of Relativity, in application to FLRW- (Friedmann-Lemaitre-Robertson-Walker) cosmological universes, leads to the conclusion that the photon wavelengths increase during their free passage through the spacetime metrics of the universe by the same factor as does the scale factor S. This appears to be a reasonable explanation for the presently observed Planckian CMB spectrum with its actual temperature of about 2.7 K, while at the time of its origin after the last scattering during the recombination phase its temperature should have been about 3000 K, at an epoch of about 380 ky after the Big Bang, when the scale of the universe S_r was smaller by roughly a factor of S/S_r = 1+z_r = 1100 compared to the present scale S = S_0 of the unive...

  6. Expanding antigen-specific regulatory networks to treat autoimmunity.

    Science.gov (United States)

    Clemente-Casares, Xavier; Blanco, Jesus; Ambalavanan, Poornima; Yamanouchi, Jun; Singha, Santiswarup; Fandos, Cesar; Tsai, Sue; Wang, Jinguo; Garabatos, Nahir; Izquierdo, Cristina; Agrawal, Smriti; Keough, Michael B; Yong, V Wee; James, Eddie; Moore, Anna; Yang, Yang; Stratmann, Thomas; Serra, Pau; Santamaria, Pere

    2016-02-25

    Regulatory T cells hold promise as targets for therapeutic intervention in autoimmunity, but approaches capable of expanding antigen-specific regulatory T cells in vivo are currently not available. Here we show that systemic delivery of nanoparticles coated with autoimmune-disease-relevant peptides bound to major histocompatibility complex class II (pMHCII) molecules triggers the generation and expansion of antigen-specific regulatory CD4(+) T cell type 1 (TR1)-like cells in different mouse models, including mice humanized with lymphocytes from patients, leading to resolution of established autoimmune phenomena. Ten pMHCII-based nanomedicines show similar biological effects, regardless of genetic background, prevalence of the cognate T-cell population or MHC restriction. These nanomedicines promote the differentiation of disease-primed autoreactive T cells into TR1-like cells, which in turn suppress autoantigen-loaded antigen-presenting cells and drive the differentiation of cognate B cells into disease-suppressing regulatory B cells, without compromising systemic immunity. pMHCII-based nanomedicines thus represent a new class of drugs, potentially useful for treating a broad spectrum of autoimmune conditions in a disease-specific manner.

  7. PLASMA TURBULENCE AND KINETIC INSTABILITIES AT ION SCALES IN THE EXPANDING SOLAR WIND

    Energy Technology Data Exchange (ETDEWEB)

    Hellinger, Petr; Trávnícek, Pavel M. [Astronomical Institute, CAS, Bocni II/1401, CZ-14100 Prague (Czech Republic); Matteini, Lorenzo [Department of Physics, Imperial College London, London SW7 2AZ (United Kingdom); Landi, Simone; Verdini, Andrea; Franci, Luca, E-mail: petr.hellinger@asu.cas.cz [Dipartimento di Fisica e Astronomia, Università degli Studi di Firenze Largo E. Fermi 2, I-50125 Firenze (Italy)

    2015-10-01

    The relationship between a decaying strong turbulence and kinetic instabilities in a slowly expanding plasma is investigated using two-dimensional (2D) hybrid expanding box simulations. We impose an initial ambient magnetic field perpendicular to the simulation box, and we start with a spectrum of large-scale, linearly polarized, random-phase Alfvénic fluctuations that have energy equipartition between kinetic and magnetic fluctuations and vanishing correlation between the two fields. A turbulent cascade rapidly develops; magnetic field fluctuations exhibit a power-law spectrum at large scales and a steeper spectrum at ion scales. The turbulent cascade leads to an overall anisotropic proton heating, protons are heated in the perpendicular direction, and, initially, also in the parallel direction. The imposed expansion leads to generation of a large parallel proton temperature anisotropy which is at later stages partly reduced by turbulence. The turbulent heating is not sufficient to overcome the expansion-driven perpendicular cooling and the system eventually drives the oblique firehose instability in a form of localized nonlinear wave packets which efficiently reduce the parallel temperature anisotropy. This work demonstrates that kinetic instabilities may coexist with strong plasma turbulence even in a constrained 2D regime.

  8. Spread spectrum image steganography.

    Science.gov (United States)

    Marvel, L M; Boncelet, C R; Retter, C T

    1999-01-01

    In this paper, we present a new method of digital steganography, entitled spread spectrum image steganography (SSIS). Steganography, which means "covered writing" in Greek, is the science of communicating in a hidden manner. Following a discussion of steganographic communication theory and review of existing techniques, the new method, SSIS, is introduced. This system hides and recovers a message of substantial length within digital imagery while maintaining the original image size and dynamic range. The hidden message can be recovered using appropriate keys without any knowledge of the original image. Image restoration, error-control coding, and techniques similar to spread spectrum are described, and the performance of the system is illustrated. A message embedded by this method can be in the form of text, imagery, or any other digital signal. Applications for such a data-hiding scheme include in-band captioning, covert communication, image tamperproofing, authentication, embedded control, and revision tracking.

  9. Raman spectrum of asphaltene

    KAUST Repository

    Abdallah, Wael A.

    2012-11-05

    Asphaltenes extracted from seven different crude oils representing different geological formations from around the globe were analyzed using the Raman spectroscopic technique. Each spectrum is fitted with four main peaks using the Gaussian function. On the basis of D1 and G bands of the Raman spectrum, asphaltene indicated an ordered structure with the presence of boundary defected edges. The average aromatic sheet size of the asphaltene molecules is estimated within the range of 1.52-1.88 nm, which represents approximately seven to eight aromatic fused rings. This estimation is based on the integrated intensity of D1 and G bands, as proposed by Tunistra and Koenig. The results here are in perfect agreement with so many other used techniques and indicate the potential applicability of Raman measurements to determine the average aromatic ring size and its boundary. © 2012 American Chemical Society.

  10. The Supersymmetric Particle Spectrum

    CERN Document Server

    Barger, V; Ohmann, P

    1994-01-01

    We examine the spectrum of supersymmetric particles predicted by grand unified theoretical (GUT) models where the electroweak symmetry breaking is accomplished radiatively. We evolve the soft supersymmetry breaking parameters according to the renormalization group equations (RGE). The minimization of the Higgs potential is conveniently described by means of tadpole diagrams. We present complete one-loop expressions for these minimization conditions, including contributions from the matter and the gauge sectors. We concentrate on the low $\\tan \\beta$ fixed point region (that provides a natural explanation of a large top quark mass) for which we find solutions to the RGE satisfying both experimental bounds and fine-tuning criteria. We also find that the constraint from the consideration of the lightest supersymmetric particle as the dark matter of the universe is accommodated in much of parameter space where the lightest neutralino is predominantly gaugino. The supersymmetric mass spectrum displays correlations...

  11. [Clinical spectrum of neuroborreliosis].

    Science.gov (United States)

    Owecki, Michał K; Kozubski, Wojciech

    2007-01-01

    Lyme disease is a multisystem infectious disease with a wide variety of symptoms involving the skin as well as nervous, musculosceletal and cardiovascular systems. Lyme disease is caused by spirochaete Borrelia burgdorferi transmitted by Ixodes tics in endemic regions. The diverse manifestations of neuroborreliosis require it to be included in differential diagnosis of many neurological disorders. The paper reviews the spectrum of clinical symptoms of nervous system involvement in early and late Lyme disease.

  12. AUTISM SPECTRUM DISORDERS (ASD)

    OpenAIRE

    Middha Akanksha; Kataria Sahil; Sandhu Premjeet; Kapoor Bhawna

    2011-01-01

    Autism or Autism Spectrum Disorders (ASD) is a serious neurological disorder affecting communication skills, social interactions, adaptability in an individual, and also causes dramatic changes in behavioral patterns. This condition typically lasts throughout one’s lifetime and affects both, children as well as adults. Research has shown a tenfold increase in autism cases over the past decade and still rising at an alarming pace. The origins of autism are not known even to modern science. Aut...

  13. Expanded mode lasers for telecommunications applications

    Science.gov (United States)

    Lealman, Ian F.

    This thesis describes the development of a long wavelength (1.55 μm) expanded mode semiconductor laser. The increased spot size of the laser improves both the coupling efficiency to cleaved fibre and fibre alignment tolerances and reduces packaging cost. In this type of device the strength of the waveguide is gradually reduced towards the front facet allowing the mode to adiabatically expand so that the laser mode is better matched in size to that of a cleaved fibre. This can be achieved by either reducing the refractive index of the guide or reducing the amount of material in the core. The structure chosen was a buried heterostructure laser that utilised a twin guide consisting of an upper higher refractive index guide (the active region of the laser) above a weak passive guide. The width of the active region was reduced along part of the device allowing the mode to expand into the weak underlying guide. The guide structure was optimised using a variable grid finite difference mode solver, and the taper length calculated by an approximation to Love's method. Detailed results are presented for the measured light-current characteristic, farfield and coupling loss to cleaved fibre. These coupling losses were compared to the calculated data thus allowing the waveguide design to be optimised. Several iterations in the design of the device were undertaken, with the aim of reducing the coupling loss to cleaved single mode fibre without significantly compromising the laser performance. The final device design had extremely low coupling losses as low as 1.2 dB to cleaved fibre. Finally, the positive impact this device had on passive alignment using a silicon motherboard is examined, and the application this technology to a range of other optoelectronic components is discussed.

  14. A new alternative to expandable pedicle screws: Expandable poly-ether-ether-ketone shell.

    Science.gov (United States)

    Demir, Teyfik

    2015-05-01

    Screw pullout is a very common problem in the fixation of sacrum with pedicle screws. The principal cause of this problem is that the cyclic micro motions in the fixation of sacrum are higher than the other regions of the vertebrae that limit the osteo-integration between bone and screw. In addition to that, the bone quality is very poor at sacrum region. This study investigated a possible solution to the pullout problem without the expandable screws' handicaps. Newly designed poly-ether-ether-ketone expandable shell and classical pedicle screws were biomechanically compared. Torsion test, pullout tests, fatigue tests, flexion/extension moment test, axial gripping capacity tests and torsional gripping capacity tests were conducted in accordance with ASTM F543, F1798 and F1717. Standard polyurethane foam and calf vertebrae were used as embedding medium for pullout tests. Classical pedicle screw pullout load on polyurethane foam was 564.8 N compared to the failure load for calf vertebrae's 1264 N. Under the same test conditions, expandable poly-ether-ether-ketone shell system's pullout loads from polyurethane foam and calf vertebrae were 1196.3 and 1890 N, respectively. The pullout values for expandable poly-ether-ether-ketone shell were 33% and 53% higher than classical pedicle screw on polyurethane foam and calf vertebrae, respectively. The expandable poly-ether-ether-ketone shell exhibited endurance on its 90% of yield load. Contrary to poly-ether-ether-ketone shell, classical pedicle screw exhibited endurance on 70% of its yield load. Expandable poly-ether-ether-ketone shell exhibited much higher pullout performance than classical pedicle screw. Fatigue performance of expandable poly-ether-ether-ketone shell is also higher than classical pedicle screw due to damping the micro motion capacity of the poly-ether-ether-ketone. Expandable poly-ether-ether-ketone shell is a safe alternative to all other expandable pedicle screw systems on mechanical perspective

  15. FOAM: Expanding the horizons of climate modeling

    Energy Technology Data Exchange (ETDEWEB)

    Tobis, M.; Foster, I.T.; Schafer, C.M. [and others

    1997-10-01

    We report here on a project that expands the applicability of dynamic climate modeling to very long time scales. The Fast Ocean Atmosphere Model (FOAM) is a coupled ocean atmosphere model that incorporates physics of interest in understanding decade to century time scale variability. It addresses the high computational cost of this endeavor with a combination of improved ocean model formulation, low atmosphere resolution, and efficient coupling. It also uses message passing parallel processing techniques, allowing for the use of cost effective distributed memory platforms. The resulting model runs over 6000 times faster than real time with good fidelity, and has yielded significant results.

  16. Two Stage Sibling Cycle Compressor/Expander.

    Science.gov (United States)

    1994-02-01

    vol. 5, p. 424. 11. L. Bauwens and M.P. Mitchell, " Regenerator Analysis: Validation of the MS*2 Stirling Cycle Code," Proc. XVIIIth International...PL-TR--94-1051 PL-TR-- 94-1051 TWO STAGE SIBLING CYCLE COMPRESSOR/EXPANDER Matthew P. Mitchell . Mitchell/ Stirling Machines/Systems, Inc. No\\ 1995...ty. THIS PAGE IS UNCLASSIFIED PL-TR-94-1051 This final report was prepared byMitchell/ Stirling Machines/Systems, Inc., Berkeley, CA under Contract

  17. Expanding Slayer Statutes to Elder Abuse.

    Science.gov (United States)

    Piel, Jennifer

    2015-09-01

    Common law has a dictum that people must not benefit from their crimes. In years past, states have enacted slayer rules to prevent killers from inheriting from their victims. The specific criteria and applicability of slayer rules vary by jurisdiction. Recently, several states, including Washington, have expanded their slayer rules to disqualify persons from inheriting if they have been involved in abuse or financial exploitation of the deceased. Reviewed herein are the abuse disinheritance laws, the relationship of the laws to concepts of testamentary capacity and undue influence, and the relevance to forensic psychiatric evaluations.

  18. Sinopec Strives to Expand Domestic Market

    Institute of Scientific and Technical Information of China (English)

    Zhang Yan

    2001-01-01

    @@ China Petrochemical Corporation (Sinopec) strives to expand its domestic market share during the Tenth Five-Year Plan period (2001~2005). The assets of Sinopec totaled 536.3 billion yuan at the end of 2000 while the business income reached 375.4billion yuan. Sinopec produced 37.24 million tons of crude oil and 3.916 billion cubic meters of natural gas and processed 105.47million tons of crude and 2.165million tons of ethylene in 2000.

  19. Expanding Lorentzian Wormholes in R2 Gravity

    Science.gov (United States)

    Sajadi, S. N.; Riazi, N.

    2011-10-01

    We present traversable, Lorentzian wormholes in the framework of R^{2} gravity. These wormholes are obtained by assuming constant Ricci scalar and trace-less equation of state. The metric is asymptotically RW, and locally that of a Lorentzian wormhole. Dynamics of the cosmological background, as well as the expanding wormhole are discussed. Weak energy condition near the wormhole throat is also examined. It is shown that the positive energy density condition can be satisfied all-over a spacelike hypersurface at large enough cosmological times and small enough, positive cosmological constant.

  20. Concurrent Manipulation of Expanded AVL Trees

    Institute of Scientific and Technical Information of China (English)

    章寅; 许卓群

    1998-01-01

    The concurrent manipulation of an expanded AVL tree(EAVL tree)s considered in this paper.The presented system can support any number of concurrent processes which perform searching,insertion and deletion on the tree.Simulation results indicate the high performance of the system.Elaborate techniques are used to achieve such a system unavailable based on any known algorithms.Methods developed in this paper may provide new insights into other problems in the area of concurrent search structure manipulation.

  1. Spectrum Services 2007

    Science.gov (United States)

    Dobos, L.; Budavári, T.; Csabai, I.; Szalay, A. S.

    2008-10-01

    We present the Filter and Spectrum Services consisting of easy-to-use web applications and web services for searching, plotting and managing large collections of spectral energy distribution data and filter profiles as well as for performing various scientific operations on spectra in a unified framework. The services provide keyword search, advanced query forms and SQL query possibilities for selecting spectra or bandpass curves which may be retrieved in a variety of file formats including XML, VOTable and ASCII. All SDSS DR1-DR5 spectra had been loaded into a database as well as the entire 2dF catalog that adds up to more than 2.5 million SEDs of about a million spatial objects, but registered users can upload their own data making it available for the rest of the community and are free to modify or delete them at any time. Theoretical catalogs, such as the Bruzual-Charlot stochastic burst model spectrum library (100k spectra) and the BaSeL stellar library are also available. Scientific services allow building rest-frame composite spectra out of selected spectra; calculating synthetic magnitudes by convolving spectra with an arbitrary set of bandpass curves of optical instrument filters to generate simulated photometric catalogs on-the-fly; galactic extinction correction, fitting of the continuum using different set of templates (Bruzual-Charlot '03 templates, SDSS eigenspectra), line fitting. All scientific functionalities are available from the web user interface and via the SOAP web services for programmers. MySpectrum is a cross-platform version of the spectrum web service for setting up your own spectrum repository. It integrates into the main service allowing easy access to your data for the whole VO community. The main idea behind our web services is to move scientific functionalities physically close to the database in order to spare network bandwidth. This way scientists may do research without setting up expensive hardware, downloading large datasets

  2. CISAPS: Complex Informational Spectrum for the Analysis of Protein Sequences

    Directory of Open Access Journals (Sweden)

    Charalambos Chrysostomou

    2015-01-01

    Full Text Available Complex informational spectrum analysis for protein sequences (CISAPS and its web-based server are developed and presented. As recent studies show, only the use of the absolute spectrum in the analysis of protein sequences using the informational spectrum analysis is proven to be insufficient. Therefore, CISAPS is developed to consider and provide results in three forms including absolute, real, and imaginary spectrum. Biologically related features to the analysis of influenza A subtypes as presented as a case study in this study can also appear individually either in the real or imaginary spectrum. As the results presented, protein classes can present similarities or differences according to the features extracted from CISAPS web server. These associations are probable to be related with the protein feature that the specific amino acid index represents. In addition, various technical issues such as zero-padding and windowing that may affect the analysis are also addressed. CISAPS uses an expanded list of 611 unique amino acid indices where each one represents a different property to perform the analysis. This web-based server enables researchers with little knowledge of signal processing methods to apply and include complex informational spectrum analysis to their work.

  3. Angular Spectrum Simulation of Pulsed Ultrasound Fields

    DEFF Research Database (Denmark)

    Du, Yigang; Jensen, Henrik; Jensen, Jørgen Arendt

    2009-01-01

    The optimization of non-linear ultrasound imaging should in a first step be based on simulation, as this makes parameter studies considerably easier than making transducer prototypes. Such a simulation program should be capable of simulating non-linear pulsed fields for arbitrary transducer...... geometries for any kind of focusing and apodization. The Angular Spectrum Approach (ASA) is capable of simulating monochromatic non-linear acoustic wave propagation. However, for ultrasound imaging the time response of each specific point in space is required, and a pulsed ASA simulation with multi temporal....... The RMS error of the pulses for all points in the simulated plane is 10.9%. The good agreement between ASA and Field II simulation for the pulsed ultrasound fields obtained in this paper makes it possible to expand Field II to non-linear pulsed fields....

  4. Hybrid spread spectrum radio system

    Science.gov (United States)

    Smith, Stephen F [London, TN; Dress, William B [Camas, WA

    2010-02-09

    Systems and methods are described for hybrid spread spectrum radio systems. A method, includes receiving a hybrid spread spectrum signal including: fast frequency hopping demodulating and direct sequence demodulating a direct sequence spread spectrum signal, wherein multiple frequency hops occur within a single data-bit time and each bit is represented by chip transmissions at multiple frequencies.

  5. Historical Notes on the Expanding Universe

    Science.gov (United States)

    Way, Michael J.; Belenkyi, Ari; Nussbaumer, Harry; Peacock, John

    2014-01-01

    The article Measuring the Hubble constant by Mario Livio and Adam Riess (Physics Today, October 2013, page 41) reviewed studies of the expanding universe from the 1920s to the present. Although the history of the subject underwent considerable compression to fit the length of a magazine article, we think it may leave a misleading impression of some of the key steps to our current understanding. We therefore offer the following clarifications. Most significantly, papers by Arthur Eddington and by Willem de Sitter in 1930, who successfully promoted Georges Lematres 1927 article for the Scientific Society of Brussels, effected a paradigm shift in interpretation of extragalactic redshifts in 1930. Before then, the astronomical community was generally unaware of the existence of nonstatic cosmological solutions and did not broadly appreciate that redshifts could be thought of locally as Doppler shifts in an expanding matter distribution. Certainly, in 1929 Edwin Hubble referred only to the de Sitter solution of 1917. At the time, the relation between distance and redshift predicted in that model was generally seen purely as a manifestation of static spacetime curvature.

  6. Expanding ester biosynthesis in Escherichia coli.

    Science.gov (United States)

    Rodriguez, Gabriel M; Tashiro, Yohei; Atsumi, Shota

    2014-04-01

    To expand the capabilities of whole-cell biocatalysis, we have engineered Escherichia coli to produce various esters. The alcohol O-acyltransferase (ATF) class of enzyme uses acyl-CoA units for ester formation. The release of free CoA upon esterification with an alcohol provides the free energy to facilitate ester formation. The diversity of CoA molecules found in nature in combination with various alcohol biosynthetic pathways allows for the biosynthesis of a multitude of esters. Small to medium volatile esters have extensive applications in the flavor, fragrance, cosmetic, solvent, paint and coating industries. The present work enables the production of these compounds by designing several ester pathways in E. coli. The engineered pathways generated acetate esters of ethyl, propyl, isobutyl, 2-methyl-1-butyl, 3-methyl-1-butyl and 2-phenylethyl alcohols. In particular, we achieved high-level production of isobutyl acetate from glucose (17.2 g l(-1)). This strategy was expanded to realize pathways for tetradecyl acetate and several isobutyrate esters.

  7. Subspace Expanders and Matrix Rank Minimization

    CERN Document Server

    Khajehnejad, Amin; Hassibi, Babak

    2011-01-01

    Matrix rank minimization (RM) problems recently gained extensive attention due to numerous applications in machine learning, system identification and graphical models. In RM problem, one aims to find the matrix with the lowest rank that satisfies a set of linear constraints. The existing algorithms include nuclear norm minimization (NNM) and singular value thresholding. Thus far, most of the attention has been on i.i.d. Gaussian measurement operators. In this work, we introduce a new class of measurement operators, and a novel recovery algorithm, which is notably faster than NNM. The proposed operators are based on what we refer to as subspace expanders, which are inspired by the well known expander graphs based measurement matrices in compressed sensing. We show that given an $n\\times n$ PSD matrix of rank $r$, it can be uniquely recovered from a minimal sampling of $O(nr)$ measurements using the proposed structures, and the recovery algorithm can be cast as matrix inversion after a few initial processing s...

  8. Unintended Consequences of Expanding the Genetic Alphabet.

    Science.gov (United States)

    Pollum, Marvin; Ashwood, Brennan; Jockusch, Steffen; Lam, Minh; Crespo-Hernández, Carlos E

    2016-09-14

    The base pair d5SICS·dNaM was recently reported to incorporate and replicate in the DNA of a modified strain of Escherichia coli, thus making the world's first stable semisynthetic organism. This newly expanded genetic alphabet may allow organisms to store considerably more information in order to translate proteins with unprecedented enzymatic activities. Importantly, however, there is currently no knowledge of the photochemical properties of d5SICS or dNaM-properties that are central to the chemical integrity of cellular DNA. In this contribution, it is shown that excitation of d5SICS or dNaM with near-visible light leads to efficient trapping of population in the nucleoside's excited triplet state in high yield. Photoactivation of these long-lived, reactive states is shown to photosensitize cells, leading to the generation of reactive oxygen species and to a marked decrease in cell proliferation, thus warning scientists of the potential phototoxic side effects of expanding the genetic alphabet.

  9. Familiarity expands space and contracts time.

    Science.gov (United States)

    Jafarpour, Anna; Spiers, Hugo

    2017-01-01

    When humans draw maps, or make judgments about travel-time, their responses are rarely accurate and are often systematically distorted. Distortion effects on estimating time to arrival and the scale of sketch-maps reveal the nature of mental representation of time and space. Inspired by data from rodent entorhinal grid cells, we predicted that familiarity to an environment would distort representations of the space by expanding the size of it. We also hypothesized that travel-time estimation would be distorted in the same direction as space-size, if time and space rely on the same cognitive map. We asked international students, who had lived at a college in London for 9 months, to sketch a south-up map of their college district, estimate travel-time to destinations within the area, and mark their everyday walking routes. We found that while estimates for sketched space were expanded with familiarity, estimates of the time to travel through the space were contracted with familiarity. Thus, we found dissociable responses to familiarity in representations of time and space. © 2016 The Authors Hippocampus Published by Wiley Periodicals, Inc.

  10. "Phase freezeout" in isentropically expanding matter

    CERN Document Server

    Iosilevskiy, Igor

    2014-01-01

    Features of isentropic expansion of warm dense matter (WDM) created by intense energy fluxes (strong shock compression or instant isochoric heating by laser or heavy ions) are under discussion in situation when ($i$) -- thermodynamic trajectory of such expansion crosses binodal of liquid-gas phase transition, and ($ii$) -- expansion within the two-phase region is going along equilibrium branch (not metastable one) of the two-phase mixture isentrope. It is known in the plane case that because of break in the expansion isentrope at binodal point (in $P-V$ plane) i.e. jump of sound velocity in this point, there appears extended zone ("boiling layer") of uniformity in expanding material with constant thermodynamic and kinematic parameters. It corresponds just to the state on this binodal of boiling liquid. The point is that because of self-similarity of such expansion (in plane case) this boiling layer contains finite and fixed part of whole expanding material. This property makes it possible (at least formally) ...

  11. Expanded HIV Screening Projected to Decrease Spread of the Virus

    Science.gov (United States)

    ... of the Virus Expanded HIV Screening Projected To Decrease Spread of the Virus Email Facebook Twitter April ... 2014, April 3). Expanded HIV Screening Projected To Decrease Spread of the Virus. Retrieved from https://www. ...

  12. Expanding Global Mindedness through a 4-H International Village

    Science.gov (United States)

    Seal, Susan D.; Peterson, Donna J.; Iwata, Chieko; Kobia, Caroline; Reddy, Raja

    2017-01-01

    With expanding global interdependence, it is vital that 4-H youths learn more about the ever-increasing diverse cultures in their own communities as well as expand their global mindedness and understanding of globalization. The 4-H International Village (a) offers a comfortable yet engaging avenue for youths to expand their knowledge of and…

  13. The Expanding Marketplace for Applied Geophysics

    Science.gov (United States)

    Carlson, N.; Sirles, P.

    2012-12-01

    While the image of geophysics for the proverbial "layman" often seems limited to volcanoes and earthquakes, and to the geoscientist this image enlarges to include oil or minerals exploration and whole earth studies, there has been a steady increase in the application of geophysics into the realm of "daily life", such as real estate deals, highway infrastructure, and flood protection. This expansion of applications can be attributed to the improved economics from advances in equipment and interpretation. Traditional geophysical methods that at one time often only fit within the budgets of oil, gas, and minerals exploration programs can now be economically applied to much smaller scale needs like contaminant mapping, landfill delineation, and levee investigations. A real-world, economic example of this expanding marketplace is our company, which began very small and was aimed almost exclusively at the minerals exploration market. Most of our growth has been in the last 10 years, when we have expanded to five offices and a staff with almost 40 geoscientist degrees (21 in geophysics); much of this growth has been in the non-oil, non-minerals arenas. While much of our work still includes minerals exploration, other projects this year include wind-farm foundation studies, cavity detection above underground nuclear tests, landfill studies, acid mine drainage problems, and leaks in evaporation ponds. A methodology example of this expanding market is the induced polarization (IP) survey, once primarily used for minerals exploration, particularly large porphyry copper deposits, but now efficient enough to also use in environmental studies. The IP method has been particularly useful in delineating and characterizing old, poorly documented landfills, and recent research suggests it may also be useful in monitoring the accelerated biodegradation processes used in some cases to rehabilitate the sites. Compared to temperature monitoring systems, IP may be more useful in providing

  14. Optical cavity resonator in an expanding universe

    Science.gov (United States)

    Kopeikin, Sergei M.

    2015-02-01

    We study the cosmological evolution of frequency of a standing electromagnetic wave in a resonant optical cavity placed to the expanding manifold described by the Robertson-Walker metric. Because of the Einstein principle of equivalence (EEP), one can find a local coordinate system (a local freely falling frame), in which spacetime is locally Minkowskian. However, due to the conformal nature of the Robertson-Walker metric the conventional transformation to the local inertial coordinates introduces ambiguity in the physical interpretation of the local time coordinate, . Therefore, contrary to a common-sense expectation, a straightforward implementation of EEP alone does not allow us to unambiguously decide whether atomic clocks based on quantum transitions of atoms, ticks at the same rate as the clocks based on electromagnetic modes of a cavity. To resolve this ambiguity we have to analyse the cavity rigidity and the oscillation of its electromagnetic modes in an expanding universe by employing the full machinery of the Maxwell equations irrespectively of the underlying theory of gravity. We proceed in this way and found out that the size of the cavity and the electromagnetic frequency experience an adiabatic drift in conformal (unphysical) coordinates as the universe expands in accordance with the Hubble law. We set up the oscillation equation for the resonant electromagnetic modes, solve it by the WKB approximation, and reduce the coordinate-dependent quantities to their counterparts measured by a local observer who counts time with atomic clock. The solution shows that there is a perfect mutual cancellation of the adiabatic drift of cavity's frequency by space transformation to local coordinates and the time counted by the clocks based on electromagnetic modes of cavity has the same rate as that of atomic clocks. We conclude that if general relativity is correct and the local expansion of space is isotropic there should be no cosmological drift of frequency of a

  15. Supernovae anisotropy power spectrum

    CERN Document Server

    Ghodsi, Hoda; Habibi, Farhang

    2016-01-01

    We contribute another anisotropy study to this field of research using Supernovae Type Ia (SNe Ia). In this work, we utilise the power spectrum calculation method and apply it to both the current SNe Ia data and simulation. Our simulations are constructed with the characteristics of the upcoming survey of the Large Synoptic Survey Telescope (LSST), which shall bring us the largest SNe Ia collection to date. We make predictions for the amplitude of a possible dipole anisotropy or anisotropy in higher multipole moments that would be detectable by the LSST.

  16. Expanding forensic science through forensic intelligence.

    Science.gov (United States)

    Ribaux, Olivier; Talbot Wright, Benjamin

    2014-12-01

    Research and Development ('R&D') in forensic science currently focuses on innovative technologies improving the efficiency of existing forensic processes, from the detection of marks and traces at the scene, to their presentation in Court. R&D approached from this perspective provides no response to doubts raised by recent criminological studies, which question the effective contribution of forensic science to crime reduction, and to policing in general. Traces (i.e. forensic case data), as remnants of criminal activity are collected and used in various forms of crime monitoring and investigation. The aforementioned doubts therefore need to be addressed by expressing how information is conveyed by traces in these processes. Modelling from this standpoint expands the scope of forensic science and provides new R&D opportunities. Twelve propositions for R&D are stated in order to pave the way.

  17. Yaglom law in the expanding solar wind

    CERN Document Server

    Gogoberidze, G; Carbone, V

    2013-01-01

    We study the Yaglom law, which relates the mixed third order structure function to the average dissipation rate of turbulence, in a uniformly expanding solar wind by using the two scales expansion model of magnetohydrodynamic (MHD) turbulence. We show that due to the expansion of the solar wind two new terms appear in the Yaglom law. The first term is related to the decay of the turbulent energy by nonlinear interactions, whereas the second term is related to the non-zero cross-correlation of the Els\\"asser fields. Using magnetic field and plasma data from WIND and Helios 2 spacecrafts, we show that at lower frequencies in the inertial range of MHD turbulence the new terms become comparable to Yaglom's third order mixed moment, and therefore they cannot be neglected in the evaluation of the energy cascade rate in the solar wind.

  18. Henry Norris Russell and the Expanding Universe

    CERN Document Server

    DeVorkin, David H

    2013-01-01

    Henry Norris Russell, one of the most influential American astronomers of the first half of the 20th Century, had a special place in his heart for the Lowell Observatory. Although privately critical of the founder for his pronouncements about life on Mars and the superiority of the Mars Hill observing site, he always supported the Observatory in public and professional circles. He staunchly supported Tombaugh's detection of a planet as leading from Lowell's prediction, and always promoted V. M. Slipher's spectroscopic investigations of planetary and stellar phenomena. But how did he react to Slipher's puzzling detection of the extreme radial velocities of spiral nebulae starting in 1912, and how did he regard the extension and interpretation of those observations by Hubble and others in following decades? Here we describe the arc of Russell's reactions, dating from Slipher's first detection, as an indicator of how mainstream stellar astronomers reacted to the concept of an expanding universe.

  19. Expandable pallet for space station interface attachments

    Science.gov (United States)

    Wesselski, Clarence J. (Inventor)

    1989-01-01

    A foldable expandable pallet having a basic square configuration is disclosed. Each pallet is comprised of a plurality of struts, joined together by node point fittings to make a rigid structure. Some of the struts have hinge fittings and are spring loaded to permit collapse of the module for stowage and transport to a space station. Dimensions of the pallet are selected to provide convenient, closely spaced attachment points between the relatively widely spaced trusses of a space station platform. A pallet is attached to a truss at four points; one close fitting hole; two oversize holes; and a slot; to allow for thermal expansion/contraction and for manufacturing tolerances. Applications of the pallet include its use in rotary or angular joints; servicing of splints; with gridded plates; as an instrument mounting bases; and as a roadbed for a Mobile Service Center (MSC).

  20. Ergodic theory of expanding thurston maps

    CERN Document Server

    Li, Zhiqiang

    2017-01-01

    Thurston maps are topological generalizations of postcritically-finite rational maps. This book provides a comprehensive study of ergodic theory of expanding Thurston maps, focusing on the measure of maximal entropy, as well as a more general class of invariant measures, called equilibrium states, and certain weak expansion properties of such maps. In particular, we present equidistribution results for iterated preimages and periodic points with respect to the unique measure of maximal entropy by investigating the number and locations of fixed points. We then use the thermodynamical formalism to establish the existence, uniqueness, and various other properties of the equilibrium state for a Holder continuous potential on the sphere equipped with a visual metric. After studying some weak expansion properties of such maps, we obtain certain large deviation principles for iterated preimages and periodic points under an additional assumption on the critical orbits of the maps. This enables us to obtain general eq...

  1. Expanding Policy Imagination in Political Economy

    DEFF Research Database (Denmark)

    Seabrooke, Leonard

    2006-01-01

    be implemented. Historical sociology provides a way to generate information about contextual constellations through two "tonics": intentional rationality and social mechanisms. With the assistance of these tonics, historical sociology widens political economy's policy imagination.......Much of the literature in political economy seeks to capture an essential insight into the evolution of political and economic systems to provide a foundation for policy advice. This article suggests that attempts to nut out the kernels of change often restrict rather than expand policy imagination...... capacity to see political, social, and economic changes that do not conform to conventional theories, as well as distorting our understanding of how the contemporary world works. What policymakers want, more than prediction or recitation of conventional theories, is context to understand how policy can...

  2. Homogeneous cosmology with aggressively expanding civilizations

    CERN Document Server

    Olson, S Jay

    2014-01-01

    In the context of a homogeneous universe, we note that the appearance of aggressively expanding advanced life is geometrically similar to the process of nucleation and bubble growth in a first-order cosmological phase transition. We exploit this similarity to describe the dynamics of life saturating the universe on a cosmic scale, adapting the phase transition model to incorporate probability distributions of expansion and resource consumption strategies. Through a series of numerical solutions covering several orders of magnitude in the input assumption parameters, the resulting cosmological model is used to address basic questions related to the intergalactic spreading of life, dealing with issues such as timescales, observability, competition between strategies, and first-mover advantage. Finally, we examine physical effects on the universe itself, such as reheating and the backreaction on the evolution of the scale factor, if such life is able to control and convert a significant fraction of the available...

  3. Particles formation in an expanding plasma

    Energy Technology Data Exchange (ETDEWEB)

    Lescoute, E.; Hallo, L.; Chimier, B.; Tikhonchuk, V.T.; Stenz, C. [Bordeaux-1 Univ., CELIA, CNRS-CEA, 33 - Talence (France); Hebert, D.; Chevalier, J.M.; Rullier, J.L.; Palmier, S. [CEA Centre d' Etudes Scientifiques et Techniques d' Aquitaine, 33 - Le Barp (France)

    2009-08-15

    Interaction of a laser beam with a target generates a high velocity expanding plasma plume, solid debris and liquid nano- and micro-particles. They are produced from plasma recombination and vapor condensation and can be deposited on optical elements located nearby the target. Two distinct kinds of particles were observed depending on the temperature achieved in the plasma plume: large micrometer-size fragments for temperatures lower than the critical temperature, and very small nanometer-size particles for higher temperatures. The paper presents experimental observations of fragments and nano-particles in plasma plumes and a comparison with models. A good agreement has been found for nano-particle sizes and distributions. This simple modeling can also be used for nuclei production in the nanosecond time scale. Our estimates show that particle size can be correlated to laser wavelength and fluences.

  4. Helical chirality induction of expanded porphyrin analogues

    Indian Academy of Sciences (India)

    Jun-Ichiro Setsune

    2012-11-01

    Expanded porphyrin analogues with unique figure-eight conformation were prepared by way of useful pyrrole intermediates such as bis(azafulvene)s and 2-borylpyrrole. Supramolecular chirogenesis of cyclooctapyrrole O1 with 32-cycloconjugation was successfully applied to determine absolute configuration of chiral carboxylic acids. Dinuclear CuII complex of cyclooctapyrrole O2 with interrupted -conjugation was resolved by HPLC into enantiomers and their helical handedness was determined by theoretical simulation of their CD spectral pattern. Enantioselective induction of helicity in the metal helicate formation in the presence of a chiral promoter was demonstrated by using ()-(+)-1-(1-phenyl)ethylamine that favoured , helicity. Dinuclear CoII complexes of cyclotetrapyrroletetrapyridine O3 were found to be substitution labile and pick up amino acid anions in water. Those amino acid complexes of O3Co2 were rendered to adopt a particular unidirectional helical conformation preferentially depending on the ligated amino acid anion.

  5. Rubella Seroprevalence Before Expanded Vaccination Program

    Directory of Open Access Journals (Sweden)

    Kenan Sener

    2007-10-01

    Full Text Available Altough rubella is usually a mild childhood disease, but when it occurs early in pregnancy are severe. In this study, seroprevalence of rubella antibodies have been invastegated in various age groups especially chilbearing age’s women before Expanded Vaccination Programme. IgM and IgG antibodies against rubella were tested by ELISA kits. Seropositivity of rubella IgG was 89,5% in chlidbearing age’s women. In Turkey, the vaccine has been on the market since 1989 but rubella vaccination is currently included in the national immunization programme. Hence, our results are important for comparison of the seroprevalence rates after national vaccination program. [TAF Prev Med Bull 2007; 6(5.000: 371-374

  6. Rubella Seroprevalence Before Expanded Vaccination Program

    Directory of Open Access Journals (Sweden)

    Kenan Sener

    2007-10-01

    Full Text Available Altough rubella is usually a mild childhood disease, but when it occurs early in pregnancy are severe. In this study, seroprevalence of rubella antibodies have been invastegated in various age groups especially chilbearing age’s women before Expanded Vaccination Programme. IgM and IgG antibodies against rubella were tested by ELISA kits. Seropositivity of rubella IgG was 89,5% in chlidbearing age’s women. In Turkey, the vaccine has been on the market since 1989 but rubella vaccination is currently included in the national immunization programme. Hence, our results are important for comparison of the seroprevalence rates after national vaccination program. [TAF Prev Med Bull. 2007; 6(5: 371-374

  7. Expanding Scope of Practice for Ontario Optometrists

    Directory of Open Access Journals (Sweden)

    Emily Bray

    2017-06-01

    Full Text Available In 2011, The Optometry Act, 1991 was amended to include The Designated Drugs and Standards of Practice Regulation which expanded the scope of practice for Ontario optometrists to include prescribing privileges from a specific list of drugs. The goals of the optometry reform were to increase access to care, decrease burden on medical and hospital resources and allow optometrists to practice to their full scope. The policy response was spurred by a recommendation from the Health Professions Regulatory Advisory Council and the prescribing precedence in other Canadian jurisdictions. Bill 171, The Health Systems Improvement Act, amended The Optometry Act, 1991, Section 4 to include the authorized act of prescribing designated drugs, while The Designated Drugs and Standards of Practice, passed on 6 April 2011, listed the individual drugs optometrists are authorized to prescribe. The resulting response of a specific list of authorized drugs was seen as a limiting and inflexible system that will require changes as newer drugs are developed.

  8. Heptaphyrins: Expanded porphyrins with seven heterocyclic rings

    Indian Academy of Sciences (India)

    Venkataramanarao G Anand; Simi K Pushpan; Sundararaman Venkatraman; Tavarekere K Chandrashekar

    2003-10-01

    Expanded porphyrins containing seven pyrrole/heterocyclic rings linked in a cyclic fashion are termed heptaphyrins. The number of -electrons in heptaphyrins depends on the number of meso carbon bridges used to link the heterocyclic rings, accordingly heptaphyrins with 28-electrons and 30 -electrons are reported to date. Both condensation reactions of the appropriate precursors and acid-catalysed oxidative coupling reactions have been utilized to synthesise the heptaphyrins. The 30 heptaphyrins exhibit rich structural diversity where some of the heterocyclic rings in the macrocycle undergo a 180° ring flipping. An overview of the synthetic methods employed for the synthesis of heptaphyrins, their spectroscopic properties, structural behaviour and aromatic properties are highlighted in this paper.

  9. Expanding Greenland’s Glacial Record

    DEFF Research Database (Denmark)

    Bjørk, Anders Anker

    Mass loss from the Greenland Ice Sheet and adjecent glaciers and ice caps has accelerated within the last decades, and these changes are accurately observed using a variety of different data products. However, the observational era is relatively short offering little insight into past dynamics. O...... the entire 20th century show rapid and widespread responses to climate change. On a longer time-scale is the Holocene history of Helheim Glacier reconstructed using evidence of glacial presence accumulated in lake sediments....... On order to expand the glacial history of Greenland, this thesis explores physical and geological archives for evidence of the glaciers’ past response to climatic variations. Using aerial photographs, the dynamic history of the Greenland Ice Sheet is extended back to 1900 C.E. Glacier changes covering...

  10. Painful Na-channelopathies: an expanding universe.

    Science.gov (United States)

    Waxman, Stephen G

    2013-07-01

    The universe of painful Na-channelopathies--human disorders caused by mutations in voltage-gated sodium channels--has recently expanded in three dimensions. We now know that mutations of sodium channels cause not only rare genetic 'model disorders' such as inherited erythromelalgia and channelopathy-associated insensitivity to pain but also common painful neuropathies. We have learned that mutations of NaV1.8, as well as mutations of NaV1.7, can cause painful Na-channelopathies. Moreover, recent studies combining atomic level structural models and pharmacogenomics suggest that the goal of genomically guided pain therapy may not be unrealistic. Copyright © 2013 Elsevier Ltd. All rights reserved.

  11. Cosmic strings in an expanding spacetime

    Energy Technology Data Exchange (ETDEWEB)

    Stein-Schabes, J.A.; Burd, A.B.

    1987-04-01

    We investigate the stability of a static, infinitely long and straight vacuum string solution under inhomogeneous axisymmetric time-dependent perturbations. We find it to be perturbatively stable. We further extend our work by finding a string solutions in an expanding Universe. The back reaction of the string on the gravitational field has been ignored. The background is assumed to be a Friedman-Robertson-Walker (FRW) cosmology. By numerically integrating the field equations in a radiation and matter dominated models, we discover oscillatory solutions. The possible damping of these oscillations is discussed. For late times the solution becomes identical to the static one studied in the first part of the paper. 19 refs., 8 figs.

  12. Expanding Greenland’s Glacial Record

    DEFF Research Database (Denmark)

    Bjørk, Anders Anker

    Mass loss from the Greenland Ice Sheet and adjecent glaciers and ice caps has accelerated within the last decades, and these changes are accurately observed using a variety of different data products. However, the observational era is relatively short offering little insight into past dynamics. O...... the entire 20th century show rapid and widespread responses to climate change. On a longer time-scale is the Holocene history of Helheim Glacier reconstructed using evidence of glacial presence accumulated in lake sediments....... On order to expand the glacial history of Greenland, this thesis explores physical and geological archives for evidence of the glaciers’ past response to climatic variations. Using aerial photographs, the dynamic history of the Greenland Ice Sheet is extended back to 1900 C.E. Glacier changes covering...

  13. Gravitational wave memory in an expanding universe

    Science.gov (United States)

    Tolish, Alexander; Wald, Robert

    2016-03-01

    We investigate the gravitational wave memory effect in an expanding FLRW spacetime. We find that if the gravitational field is decomposed into gauge-invariant scalar, vector, and tensor modes after the fashion of Bardeen, only the tensor mode gives rise to memory, and this memory can be calculated using the retarded Green's function associated with the tensor wave equation. If locally similar radiation source events occur on flat and FLRW backgrounds, we find that the resulting memories will differ only by a redshift factor, and we explore whether or not this factor depends on the expansion history of the FLRW universe. We compare our results to related work by Bieri, Garfinkle, and Yau.

  14. Roots Air Management System with Integrated Expander

    Energy Technology Data Exchange (ETDEWEB)

    Stretch, Dale [Eaton Corporation, Menomonee Falls, WI (United States); Wright, Brad [Eaton Corporation, Menomonee Falls, WI (United States); Fortini, Matt [Eaton Corporation, Menomonee Falls, WI (United States); Fink, Neal [Ballard Power Systems, Burnaby, BC (Canada); Ramadan, Bassem [Kettering Univ., Flint, MI (United States); Eybergen, William [Eaton Corporation, Menomonee Falls, WI (United States)

    2016-07-06

    PEM fuel cells remain an emerging technology in the vehicle market with several cost and reliability challenges that must be overcome in order to increase market penetration and acceptance. The DOE has identified the lack of a cost effective, reliable, and efficient air supply system that meets the operational requirements of a pressurized PEM 80kW fuel cell as one of the major technological barriers that must be overcome. This project leveraged Roots positive displacement development advancements and demonstrated an efficient and low cost fuel cell air management system. Eaton built upon its P-Series Roots positive displacement design and shifted the peak efficiency making it ideal for use on an 80kW PEM stack. Advantages to this solution include: • Lower speed of the Roots device eliminates complex air bearings present on other systems. • Broad efficiency map of Roots based systems provides an overall higher drive cycle fuel economy. • Core Roots technology has been developed and validated for other transportation applications. Eaton modified their novel R340 Twin Vortices Series (TVS) Roots-type supercharger for this application. The TVS delivers more power and better fuel economy in a smaller package as compared to other supercharger technologies. By properly matching the helix angle with the rotor’s physical aspect ratio, the supercharger’s peak efficiency can be moved to the operating range where it is most beneficial for the application. The compressor was designed to meet the 90 g/s flow at a pressure ratio of 2.5, similar in design to the P-Series 340. A net shape plastic expander housing with integrated motor and compressor was developed to significantly reduce the cost of the system. This integrated design reduced part count by incorporating an overhung expander and motor rotors into the design such that only four bearings and two shafts were utilized.

  15. Autistic spectrum disorders.

    Science.gov (United States)

    Singhania, Rajeshree

    2005-04-01

    Autistic spectrum disorders is a complex developmental disorder with social and communication dysfunction at its core. It has a wide clinical spectrum with a common triad of impairments -- social communication, social interaction and social imagination. Even mild or subtle difficulties can have a profound and devastating impact on the child. To be able to provide suitable treatments and interventions the distinctive way of thinking and learning of autistic children has to be understood. The core areas of social, emotional, communication and language deficits have to be addressed at all levels of functioning. The important goals of assessment include a categorical diagnosis of autism that looks at differential diagnosis, a refined precise documentation of the child's functioning in various developmental domains and ascertaining presence of co-morbid conditions. The interventions have to be adapted to the individual's chronological age, developmental phase and level of functioning. The strategies of curriculum delivery and teaching the child with autism is distinctive and includes presence of structure to increase predictability and strategies to reduce arousal of anxiety.

  16. Transverse myelitis spectrum disorders

    Directory of Open Access Journals (Sweden)

    Pandit Lekha

    2009-01-01

    Full Text Available Acute transverse myelitis (ATM is an inflammatory demyelinating disorder that affects the spinal cord focally resulting in motor sensory and autonomic dysfunction. Establishing the diagnosis of ATM is not as difficult as determining the possible etiology. There is a difference in the perception of ATM seen in the West as compared to developing countries. In the West multiple sclerosis (MS is the most common inflammatory disorder of the central nervous system. An attack of ATM may be the beginning of MS. However, this may not be the case in developing countries where MS is uncommon. Most often transverse myelitis is monophasic and at best represents a site-restricted form of acute disseminated encephalomyelitis (ADEM. Traditionally the combination of optic neuritis and ATM, occurring as a monophasic illness would have been called as neuromyelitis optica (NMO. Changing concepts in the definition of NMO and the discovery of a biomarker, neuromyelitis optica immunoglobulin (NMO_IgG, has changed the way relapsing autoimmune disorders are being perceived currently. A variety of idiopathic inflammatory disorders such as Japanese form of optic spinal MS, recurrent myelitis, and recurrent optic neuritis have been brought under the umbrella of neuromyelitis spectrum disorders because of the association with NMO-IgG. Complete transverse myelitis accompanied by longitudinally extensive transverse myelitis which is seronegative for this biomarker has also been reported from several countries including Japan, Australia, and India. Thus, ATM is a heterogeneous disorder with a varied clinical spectrum, etiology, and outcome.

  17. Two Expanding Integrable Models of the Geng-Cao Hierarchy

    Directory of Open Access Journals (Sweden)

    Xiurong Guo

    2014-01-01

    interesting results. In the paper, we will deduce two kinds of expanding integrable models of the Geng-Cao (GC hierarchy by constructing different 6-dimensional Lie algebras. One expanding integrable model (actually, it is a nonlinear integrable coupling reduces to a generalized Burgers equation and further reduces to the heat equation whose expanding nonlinear integrable model is generated. Another one is an expanding integrable model which is different from the first one. Finally, the Hamiltonian structures of the two expanding integrable models are obtained by employing the variational identity and the trace identity, respectively.

  18. A Cognitive Agent for Spectrum Monitoring and Informed Spectrum Access

    Science.gov (United States)

    2017-06-01

    ARL-TR-8041 ● JUNE 2017 US Army Research Laboratory A Cognitive Agent for Spectrum Monitoring and Informed Spectrum Access by...needed. Do not return it to the originator. ARL-TR-8041 ● JUNE 2017 US Army Research Laboratory A Cognitive Agent for Spectrum...should be aware that notwithstanding any other provision of law, no person shall be subject to any penalty for failing to comply with a collection of

  19. Sakurai's Object Continues to Brighten and Expand

    Science.gov (United States)

    Hinkle, Kenneth H.; Joyce, Richard R.; Matheson, Thomas

    2017-01-01

    Sakurai's Object (V4334 Sgr), the prototype final flash object discovered in the mid-1990s, was observed to undergo rapid cooling becoming as faint as 25th magnitude at K during the first decade of the 21st century. A review of imaging data suggests the minimum K magnitude occurred about 2006. Sakuarai's Object was re-acquired at K in 2010. Between 2010 Sep and 2013 Apr Sakurai's object brightened more than 2 magnitudes to K=14.2. Here we report on a Gemini-NIRI K band AO image obtained in 2016 July. The Ks magnitude was 13.35. The AO image also records the continuing expansion of the debris cloud. The central star remains obscured. Spectro-spatial NIFS images of the spectral region around He I 1.0830 micron and a GMOS optical spectrum, both observed in 2015, will also be displayed.

  20. Rotational spectrum of tryptophan

    Energy Technology Data Exchange (ETDEWEB)

    Sanz, M. Eugenia, E-mail: maria.sanz@kcl.ac.uk; Cabezas, Carlos, E-mail: ccabezas@qf.uva.es; Mata, Santiago, E-mail: santiago.mata@uva.es; Alonso, Josè L., E-mail: jlalonso@qf.uva.es [Grupo de Espectroscopia Molecular (GEM), Edificio Quifima, Laboratorios de Espectroscopia y Bioespectroscopia, Unidad Asociada CSIC, Parque Científico Uva, Universidad de Valladolid, 47011 Valladolid (Spain)

    2014-05-28

    The rotational spectrum of the natural amino acid tryptophan has been observed for the first time using a combination of laser ablation, molecular beams, and Fourier transform microwave spectroscopy. Independent analysis of the rotational spectra of individual conformers has conducted to a definitive identification of two different conformers of tryptophan, with one of the observed conformers never reported before. The analysis of the {sup 14}N nuclear quadrupole coupling constants is of particular significance since it allows discrimination between structures, thus providing structural information on the orientation of the amino group. Both observed conformers are stabilized by an O–H···N hydrogen bond in the side chain and a N–H···π interaction forming a chain that reinforce the strength of hydrogen bonds through cooperative effects.

  1. Rotational Spectrum of Tryptophan

    Science.gov (United States)

    Sanz, M. Eugenia; Cabezas, Carlos; Mata, Santiago; Alonso, José L.

    2014-06-01

    The rotational spectrum of the natural amino acid tryptophan has been observed using a recently constructed LA-MB-FTMW spectrometer, specifically designed to optimize the detection of heavier molecules at a lower frequency range. Independent analyses of the rotational spectra of individual conformers have conducted to a definitive identification of two different conformers of tryptophan, with one of the observed conformers never reported before. The experimental values of the 14N nuclear quadrupole coupling constants have been found capital in the discrimination of the conformers. Both observed conformers are stabilized by a O-H\\cdotsN hydrogen bond in the side chain and a N-H\\cdotsπ interaction forming a chain that reinforces the strength of hydrogen bonds through cooperative effects.

  2. Autism Spectrum Disorders

    Directory of Open Access Journals (Sweden)

    Rebecca E. Rosenberg

    2011-01-01

    Full Text Available We used a national online registry to examine variation in cumulative prevalence of community diagnosis of psychiatric comorbidity in 4343 children with autism spectrum disorders (ASD. Adjusted multivariate logistic regression models compared influence of individual, family, and geographic factors on cumulative prevalence of parent-reported anxiety disorder, depression, bipolar disorder, and attention deficit/hyperactivity disorder or attention deficit disorder. Adjusted odds of community-assigned lifetime psychiatric comorbidity were significantly higher with each additional year of life, with increasing autism severity, and with Asperger syndrome and pervasive developmental disorder—not otherwise specified compared with autistic disorder. Overall, in this largest study of parent-reported community diagnoses of psychiatric comorbidity, gender, autistic regression, autism severity, and type of ASD all emerged as significant factors correlating with cumulative prevalence. These findings could suggest both underlying trends in actual comorbidity as well as variation in community interpretation and application of comorbid diagnoses in ASD.

  3. Electromagnetic spectrum management system

    Science.gov (United States)

    Seastrand, Douglas R.

    2017-01-31

    A system for transmitting a wireless countermeasure signal to disrupt third party communications is disclosed that include an antenna configured to receive wireless signals and transmit wireless counter measure signals such that the wireless countermeasure signals are responsive to the received wireless signals. A receiver processes the received wireless signals to create processed received signal data while a spectrum control module subtracts known source signal data from the processed received signal data to generate unknown source signal data. The unknown source signal data is based on unknown wireless signals, such as enemy signals. A transmitter is configured to process the unknown source signal data to create countermeasure signals and transmit a wireless countermeasure signal over the first antenna or a second antenna to thereby interfere with the unknown wireless signals.

  4. Microwave Spectrum of Hexafluoroisopropanol

    Science.gov (United States)

    Shahi, Abhishek; Mani, Devendra; Arunan, E.

    2012-06-01

    Hexafluoroisopropanol (HFIP) is an important organic solvent and probably the only solvent which can dissolve polythene. IR studies, on this molecule confirm the existence of antiperiplanar (ap) and synclinical (sc) conformers. We have observed pure rotational spectrum of this molecule and the fitted rotational constants (A= 2105.1208(11) MHz, B= 1053.9942(3) MHz, C= 932.3398(3) MHz) confirm the presence of ap conformer. There are many other observed lines which most probably corresponds to sc structure and due to the large amplitude motion of H-atom, some of these transitions show tunneling splitting. Work is in progress for the deuterated (OD) and C-13 isotopologues of the monomer. HFIP is expected to exhibit interesting hydrogen bonding properties and we are planning to investigate them by studying its complex with water. The results will be presented in this talk. H. Schaal, T. Höber, and M. A. Suhm, J. Phys. Chem. A 104, 265 (2000).

  5. BOOK REVIEW: The Artful Universe Expanded

    Science.gov (United States)

    Bassett, B. A.

    2005-07-01

    The cosmos is an awfully big place and there is no better guide to its vast expanse and fascinating nooks and crannies than John Barrow. A professor of mathematical sciences at Cambridge University, Barrow embodies that rare combination of highly polished writer and expert scientist. His deft touch brings together the disparate threads of human knowledge and weaves them into a tapestry as rich and interesting for the expert as it is for the layperson. The Artful Universe Expanded is an updated edition of this popular book first published in 1995. It explores the deeply profound manner in which natural law and the nature of the cosmos have moulded and shaped us, our cultures and the very form of our arts and music—a new type of `cosmic' anthropology. The main themes Barrow chooses for revealing this new anthropology are the subjects of evolution, the size of things, the heavens and the nature of music. The book is a large, eclectic repository of knowledge often unavailable to the layperson,\\endcolumn hidden in esoteric libraries around the world. It rivals The Da Vinci Code for entertainment value and insights, but this time it is Nature’s code that is revealed. It is rare indeed to find common threads drawn through topics as diverse as The Beetles, Bach and Beethoven or between Jackson Pollock, the Aztecs, Kant, Picasso, Byzantine mosaics, uranium-235 and the helix nebula. Barrow unerringly binds them together, presenting them in a stimulating, conversational style that belies the amount of time that must have gone into researching this book. Dip into it at random, or read it from cover to cover, but do read it. The Artful Universe Expanded is an entertaining antidote to the oft-lamented pressures to know more and more about less and less and the apparently inexorable march of specialization. On reading this book one can, for a short time at least, hold in one’s mind a vision that unifies science, art and culture and glimpse a universal tapestry of great

  6. Reliability and Validity of Parent- and Child-Rated Anxiety Measures in Autism Spectrum Disorder

    Science.gov (United States)

    Kaat, Aaron J.; Lecavalier, Luc

    2015-01-01

    Autism spectrum disorder (ASD) and anxiety frequently co-occur. Research on the phenomenology and treatment of anxiety in ASD is expanding, but is hampered by the lack of instruments validated for this population. This study evaluated the self- and parent-reported Revised Child Anxiety and Depression Scale and the Multidimensional Anxiety Scale in…

  7. Dysferlin Gene Mutation Spectrum in a Large Cohort of Chinese Patients with Dysferlinopathy

    Directory of Open Access Journals (Sweden)

    Su-Qin Jin

    2016-01-01

    Conclusions: DYSF mutations in Chinese patients clustered in the N-terminal region of the gene. Exonic rearrangements were found in 23% of patients with only one pathogenic mutation identified by Sanger sequencing or NGS. The novel mutations found in this study greatly expanded the mutational spectrum of dysferlinopathy.

  8. Transition to College and Students with High Functioning Autism Spectrum Disorder: Strategy Considerations for School Counselors

    Science.gov (United States)

    Dipeolu, Abiola O.; Storlie, Cassandra; Johnson, Carol

    2014-01-01

    There are limited school counseling resources that address the unique post high school transition issues faced by students with High-functioning Autism Spectrum Disorder (HASD). While many school counselors have excellent skills in assessment, advising, and career planning, it is worthwhile to expand these to include working with students with…

  9. Reliability and Validity of Parent- and Child-Rated Anxiety Measures in Autism Spectrum Disorder

    Science.gov (United States)

    Kaat, Aaron J.; Lecavalier, Luc

    2015-01-01

    Autism spectrum disorder (ASD) and anxiety frequently co-occur. Research on the phenomenology and treatment of anxiety in ASD is expanding, but is hampered by the lack of instruments validated for this population. This study evaluated the self- and parent-reported Revised Child Anxiety and Depression Scale and the Multidimensional Anxiety Scale in…

  10. Stoppage in Autism Spectrum Disorders

    DEFF Research Database (Denmark)

    Grønborg, Therese Koops; Hansen, Stefan Nygaard; Nielsen, Svend V

    2015-01-01

    of bias in sibling recurrence risk estimation. This study investigated whether stoppage occurs in Danish families with a firstborn child diagnosed with autism spectrum disorders, and if stoppage was differential. We found that stoppage occurs moderately in Danish families affected by autism spectrum...... disorders, and that stoppage is differential. However, differential stoppage is a minor source of estimation bias in Danish sibling recurrence risk studies of autism spectrum disorders....

  11. Compressor-expander units for mobile fuel cell systems; Verdichter und Expander fuer mobile Brennstoffzellensysteme

    Energy Technology Data Exchange (ETDEWEB)

    Lang, O. [FEV Motorentechnik GmbH, Aachen (Germany).; Pischinger, S.; Schoenfelder, C.; Steidten, T. [RWTH Aachen (DE). Lehrstuhl fuer Verbrennungskraftmaschinen (VKA)

    2004-08-01

    The air supply of hydrogen-powered fuel cell systems containing PEM fuel cells has a significant impact on their efficiency and mode of operation. Therefore, several mechanical compressors and expanders were examined at the RWTH Aachen. Simulations were then performed to determine the behaviour of the fuel cell system. This article discusses the findings of a project commissioned by the Forschungsvereinigung Verbrennungskraftmaschinen e.V. (FVV). (orig.)

  12. Regression in autistic spectrum disorders.

    Science.gov (United States)

    Stefanatos, Gerry A

    2008-12-01

    A significant proportion of children diagnosed with Autistic Spectrum Disorder experience a developmental regression characterized by a loss of previously-acquired skills. This may involve a loss of speech or social responsitivity, but often entails both. This paper critically reviews the phenomena of regression in autistic spectrum disorders, highlighting the characteristics of regression, age of onset, temporal course, and long-term outcome. Important considerations for diagnosis are discussed and multiple etiological factors currently hypothesized to underlie the phenomenon are reviewed. It is argued that regressive autistic spectrum disorders can be conceptualized on a spectrum with other regressive disorders that may share common pathophysiological features. The implications of this viewpoint are discussed.

  13. Helical Screw Expander Evaluation Project. Final report

    Energy Technology Data Exchange (ETDEWEB)

    McKay, R.

    1982-03-01

    A functional 1-MW geothermal electric power plant that featured a helical screw expander was produced and then tested in Utah in 1978 to 1979 with a demonstrated average performance of approximately 45% machine efficiency over a wide range of test conditions in noncondensing operation on two-phase geothermal fluids. The Project also produced a computer-equipped data system, an instrumentation and control van, and a 1000-kW variable load bank, all integrated into a test array designed for operation at a variety of remote test sites. Additional testing was performed in Mexico in 1980 under a cooperative test program using the same test array, and machine efficiency was measured at 62% maximum with the rotors partially coated with scale, compared with approximately 54% maximum in Utah with uncoated rotors, confirming the importance of scale deposits within the machine on performance. Data are presented for the Utah testing and for the noncondensing phases of the testing in Mexico. Test time logged was 437 hours during the Utah tests and 1101 hours during the Mexico tests.

  14. Expanding the genetic heterogeneity of intellectual disability

    KAUST Repository

    Anazi, Shams

    2017-09-22

    Intellectual disability (ID) is a common morbid condition with a wide range of etiologies. The list of monogenic forms of ID has increased rapidly in recent years thanks to the implementation of genomic sequencing techniques. In this study, we describe the phenotypic and genetic findings of 68 families (105 patients) all with novel ID-related variants. In addition to established ID genes, including ones for which we describe unusual mutational mechanism, some of these variants represent the first confirmatory disease-gene links following previous reports (TRAK1, GTF3C3, SPTBN4 and NKX6-2), some of which were based on single families. Furthermore, we describe novel variants in 14 genes that we propose as novel candidates (ANKHD1, ASTN2, ATP13A1, FMO4, MADD, MFSD11, NCKAP1, NFASC, PCDHGA10, PPP1R21, SLC12A2, SLK, STK32C and ZFAT). We highlight MADD and PCDHGA10 as particularly compelling candidates in which we identified biallelic likely deleterious variants in two independent ID families each. We also highlight NCKAP1 as another compelling candidate in a large family with autosomal dominant mild intellectual disability that fully segregates with a heterozygous truncating variant. The candidacy of NCKAP1 is further supported by its biological function, and our demonstration of relevant expression in human brain. Our study expands the locus and allelic heterogeneity of ID and demonstrates the power of positional mapping to reveal unusual mutational mechanisms.

  15. Expanding the role of internal facility assessments

    Energy Technology Data Exchange (ETDEWEB)

    Kolpa, R.L.; Levenson, J.B. [Argonne National Lab., IL (United States); Weaver, M.A. [Headquarters Air Force Materiel Command, Wright-Patterson AFB, OH (United States)

    1996-05-01

    The US Air Force (USAF) Environmental Compliance Assessment and Management Program (ECAMP) is an effective and comprehensive system to evaluate environmental compliance at individual USAF installations. The ECAMP assessment is typically performed by a team of experts from the installation`s Major Command (MAJCOM) Headquarters, and is often augmented with technical contractor support. As directed by Air Force policy, an external ECAMP assessment is required at a minimum of every three years for each installation. In the intervening years, each installation is required to perform an internal ECAMP assessment, with its own personnel and resources. Even though team composition differs, the internal and external ECAMP assessments are likely to be very similar in scope, objectives, and deliverables. For over nine years, Argonne National Laboratory (ANL) has supported several Air Force MAJCOMs in performing their external ECAMP assessments. More recently, ANL has also had the opportunity to provide technical support and training at individual installations during their preparation and conduct of internal ECAMP assessments. From that experience, the authors have learned that the quality and value of the internal assessment is enhanced by making it a vehicle for training, planning, and interaction among organizations. Various strategies and techniques have been successfully employed to derive maximum benefit and insight from the internal assessment process. Experiences that involve expanding the scope and objectives of internal assessments to meet specific goals are presented. The expansion of scope and objectives include preassessment training, planning, and evaluator interactions as part of the overall internal assessment process.

  16. Expandable Purge Chambers Would Protect Cryogenic Fittings

    Science.gov (United States)

    Townsend, Ivan I., III

    2004-01-01

    Expandable ice-prevention and cleanliness-preservation (EIP-CP) chambers have been proposed to prevent the accumulation of ice or airborne particles on quick-disconnect (QD) fittings, or on ducts or tubes that contain cryogenic fluids. In the original application for which the EIP-CP chambers were conceived, there is a requirement to be able to disconnect and reconnect the QD fittings in rapid succession. If ice were to form on the fittings by condensation and freezing of airborne water vapor on the cold fitting surfaces, the ice could interfere with proper mating of the fittings, making it necessary to wait an unacceptably long time for the ice to thaw before attempting reconnection. By keeping water vapor away from the cold fitting surfaces, the EIP-CP chambers would prevent accumulation of ice, preserving the ability to reconnect as soon as required. Basically, the role of an EIP-CP chamber would be to serve as an enclosure for a flow of dry nitrogen gas that would keep ambient air away from QD cryogenic fittings. An EIP-CP chamber would be an inflatable device made of a fabriclike material. The chamber would be attached to an umbilical plate holding a cryogenic QD fitting.

  17. The expanding fireball of Nova Delphini 2013.

    Science.gov (United States)

    Schaefer, G H; ten Brummelaar, T; Gies, D R; Farrington, C D; Kloppenborg, B; Chesneau, O; Monnier, J D; Ridgway, S T; Scott, N; Tallon-Bosc, I; McAlister, H A; Boyajian, T; Maestro, V; Mourard, D; Meilland, A; Nardetto, N; Stee, P; Sturmann, J; Vargas, N; Baron, F; Ireland, M; Baines, E K; Che, X; Jones, J; Richardson, N D; Roettenbacher, R M; Sturmann, L; Turner, N H; Tuthill, P; van Belle, G; von Braun, K; Zavala, R T; Banerjee, D P K; Ashok, N M; Joshi, V; Becker, J; Muirhead, P S

    2014-11-13

    A classical nova occurs when material accreting onto the surface of a white dwarf in a close binary system ignites in a thermonuclear runaway. Complex structures observed in the ejecta at late stages could result from interactions with the companion during the common-envelope phase. Alternatively, the explosion could be intrinsically bipolar, resulting from a localized ignition on the surface of the white dwarf or as a consequence of rotational distortion. Studying the structure of novae during the earliest phases is challenging because of the high spatial resolution needed to measure their small sizes. Here we report near-infrared interferometric measurements of the angular size of Nova Delphini 2013, starting one day after the explosion and continuing with extensive time coverage during the first 43 days. Changes in the apparent expansion rate can be explained by an explosion model consisting of an optically thick core surrounded by a diffuse envelope. The optical depth of the ejected material changes as it expands. We detect an ellipticity in the light distribution, suggesting a prolate or bipolar structure that develops as early as the second day. Combining the angular expansion rate with radial velocity measurements, we derive a geometric distance to the nova of 4.54 ± 0.59 kiloparsecs from the Sun.

  18. Expanding solvent SAGD in heavy oil reservoirs

    Energy Technology Data Exchange (ETDEWEB)

    Govind, P.A. [Society of Petroleum Engineers, Canadian Section, Calgary, AB (Canada)]|[ConocoPhillips Canada Resources Corp., Calgary, AB (Canada); Das, S.; Wheeler, T.J. [Society of Petroleum Engineers, Richardson, TX (United States)]|[ConocoPhillips Co., Houston, TX (United States); Srinivasan, S. [Society of Petroleum Engineers, Richardson, TX (United States)]|[Texas Univ., Austin, TX (United States)

    2008-10-15

    Steam assisted gravity drainage (SAGD) projects have proven effective for the recovery of oil and bitumen. Expanding solvent (ES) SAGD pilot projects have also demonstrated positive results of improved performance. This paper presented the results of a simulation study that investigated several important factors of the ES-SAGD process, including solvent types; concentration; operating pressure; and injection strategy. The objectives of the study were to examine the effectiveness of the ES-SAGD process in terms of production acceleration and energy requirements; to optimize solvent selection; to understand the effect of dilation in unconsolidated oil sands and the directional impact on reservoir parameters and oil production rate in ES-SAGD; and to understand the impact of operating conditions such as pressure, solvent concentration, circulation preheating period and the role of conduction heating and grid size in this process. The advantages of ES-SAGD over SAGD were also outlined. The paper presented results of sensitivity studies that were conducted on these four factors. Conclusions and recommendations for operating strategy were also offered. It was concluded that dilation is an important factor for SAGD performance at high operating pressure. 8 refs., 15 figs.

  19. Expanded polylactide bead foaming - A new technology

    Science.gov (United States)

    Nofar, M.; Ameli, A.; Park, C. B.

    2015-05-01

    Bead foaming technology with double crystal melting peak structure has been recognized as a promising method to produce low-density foams with complex geometries. During the molding stage of the bead foams, the double peak structure generates a strong bead-to-bead sintering and maintains the overall foam structure. During recent years, polylactide (PLA) bead foaming has been of the great interest of researchers due to its origin from renewable resources and biodegradability. However, due to the PLA's low melt strength and slow crystallization kinetics, the attempts have been limited to the manufacturing methods used for expanded polystyrene. In this study, for the first time, we developed microcellular PLA bead foams with double crystal melting peak structure. Microcellular PLA bead foams were produced with expansion ratios and average cell sizes ranging from 3 to 30-times and 350 nm to 15 µm, respectively. The generated high melting temperature crystals during the saturation significantly affected the expansion ratio and cell density of the PLA bead foams by enhancing the PLA's poor melt strength and promoting heterogeneous cell nucleation around the crystals.

  20. Filtration application from recycled expanded polystyrene.

    Science.gov (United States)

    Shin, C

    2006-10-01

    Water-in-oil emulsion with drop size less than 100 mum is difficult to separate. Coalescence filtration is economical and effective for separation of secondary dispersions. Coalescence performance depends on flow rate, bed depth, fiber surface properties, and drop size. The amount of surface area of the fibers directly affects the efficiency. A new recycling method was investigated in the previous work in which polystyrene (PS) sub-mum fibers were electro-spun from recycled expanded polystyrene (EPS). These fibers are mixed with micro glass fibers to modify the glass fiber filter media. The filter media are tested in the separation of water droplets from an emulsion of water droplets in oil. The experimental results in this work show that adding nanofibers to conventional micron sized fibrous filter media improves the separation efficiency of the filter media but also increases the pressure drop. An optimum in the performance occurs (significant increase in efficiency with minimal increase in pressure drop) with the addition of about 4% by mass of 500 nm diameter PS nanofibers to glass fibers for the filters.

  1. The expanding universe of prion diseases.

    Directory of Open Access Journals (Sweden)

    Joel C Watts

    2006-03-01

    Full Text Available Prions cause fatal and transmissible neurodegenerative disease. These etiological infectious agents are formed in greater part from a misfolded cell-surface protein called PrP(C. Several mammalian species are affected by the diseases, and in the case of "mad cow disease" (BSE the agent has a tropism for humans, with negative consequences for agribusiness and public health. Unfortunately, the known universe of prion diseases is expanding. At least four novel prion diseases--including human diseases variant Creutzfeldt-Jakob disease (vCJD and sporadic fatal insomnia (sFI, bovine amyloidotic spongiform encephalopathy (BASE, and Nor98 of sheep--have been identified in the last ten years, and chronic wasting disease (CWD of North American deer (Odocoileus Specis and Rocky Mountain elk (Cervus elaphus nelsoni is undergoing a dramatic spread across North America. While amplification (BSE and dissemination (CWD, commercial sourcing of cervids from the wild and movement of farmed elk can be attributed to human activity, the origins of emergent prion diseases cannot always be laid at the door of humankind. Instead, the continued appearance of new outbreaks in the form of "sporadic" disease may be an inevitable outcome in a situation where the replicating pathogen is host-encoded.

  2. The expanding universe of prion diseases.

    Directory of Open Access Journals (Sweden)

    2006-03-01

    Full Text Available Prions cause fatal and transmissible neurodegenerative disease. These etiological infectious agents are formed in greater part from a misfolded cell-surface protein called PrP(C. Several mammalian species are affected by the diseases, and in the case of "mad cow disease" (BSE the agent has a tropism for humans, with negative consequences for agribusiness and public health. Unfortunately, the known universe of prion diseases is expanding. At least four novel prion diseases-including human diseases variant Creutzfeldt-Jakob disease (vCJD and sporadic fatal insomnia (sFI, bovine amyloidotic spongiform encephalopathy (BASE, and Nor98 of sheep-have been identified in the last ten years, and chronic wasting disease (CWD of North American deer (Odocoileus Specis and Rocky Mountain elk (Cervus elaphus nelsoni is undergoing a dramatic spread across North America. While amplification (BSE and dissemination (CWD, commercial sourcing of cervids from the wild and movement of farmed elk can be attributed to human activity, the origins of emergent prion diseases cannot always be laid at the door of humankind. Instead, the continued appearance of new outbreaks in the form of "sporadic" disease may be an inevitable outcome in a situation where the replicating pathogen is host-encoded.

  3. Physical expander in Virtual Tree Overlay

    CERN Document Server

    Izumi, Taisuke; Valero, Mathieu

    2011-01-01

    In this paper, we propose a new construction of constantdegree expanders motivated by their application in P2P overlay networks and in particular in the design of robust trees overlay. Our key result can be stated as follows. Consider a complete binary tree T and construct a random pairing {\\Pi} between leaf nodes and internal nodes. We prove that the graph G\\Pi obtained from T by contracting all pairs (leaf-internal nodes) achieves a constant node expansion with high probability. The use of our result in improving the robustness of tree overlays is straightforward. That is, if each physical node participating to the overlay manages a random pair that couples one virtual internal node and one virtual leaf node then the physical-node layer exhibits a constant expansion with high probability. We encompass the difficulty of obtaining this random tree virtualization by proposing a local, selforganizing and churn resilient uniformly-random pairing algorithm with O(log2 n) running time. Our algorithm has the merit ...

  4. Neuroimaging of Parkinson's disease: Expanding views.

    Science.gov (United States)

    Weingarten, Carol P; Sundman, Mark H; Hickey, Patrick; Chen, Nan-kuei

    2015-12-01

    Advances in molecular and structural and functional neuroimaging are rapidly expanding the complexity of neurobiological understanding of Parkinson's disease (PD). This review article begins with an introduction to PD neurobiology as a foundation for interpreting neuroimaging findings that may further lead to more integrated and comprehensive understanding of PD. Diverse areas of PD neuroimaging are then reviewed and summarized, including positron emission tomography, single photon emission computed tomography, magnetic resonance spectroscopy and imaging, transcranial sonography, magnetoencephalography, and multimodal imaging, with focus on human studies published over the last five years. These included studies on differential diagnosis, co-morbidity, genetic and prodromal PD, and treatments from L-DOPA to brain stimulation approaches, transplantation and gene therapies. Overall, neuroimaging has shown that PD is a neurodegenerative disorder involving many neurotransmitters, brain regions, structural and functional connections, and neurocognitive systems. A broad neurobiological understanding of PD will be essential for translational efforts to develop better treatments and preventive strategies. Many questions remain and we conclude with some suggestions for future directions of neuroimaging of PD. Copyright © 2015 Elsevier Ltd. All rights reserved.

  5. Expanding Taylor bubble under constant heat flux

    Science.gov (United States)

    Voirand, Antoine; Benselama, Adel M.; Ayel, Vincent; Bertin, Yves

    2016-09-01

    Modelization of non-isothermal bubbles expanding in a capillary, as a contribution to the understanding of the physical phenomena taking place in Pulsating Heat Pipes (PHPs), is the scope of this paper. The liquid film problem is simplified and solved, while the thermal problem takes into account a constant heat flux density applied at the capillary tube wall, exchanging with the liquid film surrounding the bubble and also with the capillary tube outside medium. The liquid slug dynamics is solved using the Lucas-Washburn equation. Mass and energy balance on the vapor phase allow governing equations of bubble expansion to be written. The liquid and vapor phases are coupled only through the saturation temperature associated with the vapor pressure, assumed to be uniform throughout the bubble. Results show an over-heating of the vapor phase, although the particular thermal boundary condition used here always ensures an evaporative mass flux at the liquid-vapor interface. Global heat exchange is also investigated, showing a strong decreasing of the PHP performance to convey heat by phase change means for large meniscus velocities.

  6. Spectrum handoff algorithm with imperfect spectrum sensing incognitive radio networks

    Science.gov (United States)

    Ma, Bin; Bao, Xiao-min; Xie, Xian-zhong

    2016-10-01

    To guarantee the quality of service of licensed users, the unlicensed users must vacate the occupied channel by spectrum handoff if licensed users appear in the licensed spectrum. However, spectrum sensing with false alarm and missed detection may lead to more inaccuracy problems when unlicensed users perform spectrum handoff. To improve the problems mentioned before, we utilize PRP M/G/1 queuing network model to propose a spectrum handoff algorithm with error data retransmission mechanism and analyze its performance by transmission delay and extended data delivery time. In addition, we discuss the relationship between false alarm probability, extended data delivery time and user traffic load and verify the effectiveness of the proposed algorithm.

  7. Methodological innovations expand the safety pharmacology horizon.

    Science.gov (United States)

    Pugsley, M K; Curtis, M J

    2012-09-01

    Almost uniquely in pharmacology, drug safety assessment is driven by the need for elaboration and validation of methods for detecting drug actions. This is the 9th consecutive year that the Journal of Pharmacological and Toxicological Methods (JPTM) has published themed issues arising from the annual meeting of the Safety Pharmacology Society (SPS). The SPS is now past its 10th year as a distinct (from pharmacology to toxicology) discipline that integrates safety pharmacologists from industry with those in academia and the various global regulatory authorities. The themes of the 2011 meeting were (i) the bridging of safety assessment of a new chemical entity (NCE) between all the parties involved, (ii) applied technologies and (iii) translation. This issue of JPTM reflects these themes. The content is informed by the regulatory guidance documents (S7A and S7B) that apply prior to first in human (FIH) studies, which emphasize the importance of seeking model validation. The manuscripts encompass a broad spectrum of safety pharmacology topics including application of state-of-the-art techniques for study conduct and data processing and evaluation. This includes some exciting novel integrated core battery study designs, refinements in hemodynamic assessment, arrhythmia analysis algorithms, and additionally an overview of safety immunopharmacology, and a brief survey discussing similarities and differences in business models that pharmaceutical companies employ in safety pharmacology, together with SPS recommendations on 'best practice' for the conduct of a non-clinical cardiovascular assessment of a NCE.

  8. Spherical Accretion in a Uniformly Expanding Universe

    Science.gov (United States)

    Colpi, Monica; Shapiro, Stuart L.; Wasserman, Ira

    1996-10-01

    We consider spherically symmetric accretion of material from an initially homogeneous, uniformly expanding medium onto a Newtonian point mass M. The gas is assumed to evolve adiabatically with a constant adiabatic index F, which we vary over the range Γ ɛ [1, 5/3]. We use a one-dimensional Lagrangian code to follow the spherical infall of material as a function of time. Outflowing shells gravitationally bound to the point mass fall back, giving rise to a inflow rate that, after a rapid rise, declines as a power law in time. If there were no outflow initially, Bondi accretion would result, with a characteristic accretion time-scale ta,0. For gas initially expanding at a uniform rate, with a radial velocity U = R/t0 at radius R, the behavior of the flow at all subsequent times is determined by ta,0/t0. If ta,0/t0 ≫ 1, the gas has no time to respond to pressure forces, so the fluid motion is nearly collisionless. In this case, only loosely bound shells are influenced by pressure gradients and are pushed outward. The late-time evolution of the mass accretion rate Mdot is close to the result for pure dust, and we develop a semianalytic model that accurately accounts for the small effect of pressure gradients in this limit. In the opposite regime, ta,0/t0 ≪ 1, pressure forces significantly affect the motion of the gas. At sufficiently early times, t ≤ ttr, the flow evolved along a sequence of quasi-stationary, Bondi-like states, with a time-dependent Mdot determined by the slowly varying gas density at large distances. However, at later times, t ≥ ttr, the fluid flow enters a dustllke regime; ttr is the time when the instantaneous Bondi accretion radius reaches the marginally bound radius. The transition time ttr depends sensitively on ta,0/t0 for a given Γ and can greatly exceed t0. We show that there exists a critical value Γ = 11/9, below which the transition from fluid to ballistic motion disappears. As one application of our calculations, we consider the

  9. Expanding on Successful Concepts, Models, and Organization

    Energy Technology Data Exchange (ETDEWEB)

    Teeguarden, Justin G.; Tan, Yu-Mei; Edwards, Stephen W.; Leonard, Jeremy A.; Anderson, Kim A.; Corley, Richard A.; Kile, Molly L.; L. Massey Simonich, Staci; Stone, David; Tanguay, Robert L.; Waters, Katrina M.; Harper, Stacey L.; Williams, David E.

    2016-09-06

    In her letter to the editor1 regarding our recent Feature Article “Completing the Link between Exposure Science and Toxicology for Improved Environmental Health Decision Making: The Aggregate Exposure Pathway Framework” 2, Dr. von Göetz expressed several concerns about terminology, and the perception that we propose the replacement of successful approaches and models for exposure assessment with a concept. We are glad to have the opportunity to address these issues here. If the goal of the AEP framework was to replace existing exposure models or databases for organizing exposure data with a concept, we would share Dr. von Göetz concerns. Instead, the outcome we promote is broader use of an organizational framework for exposure science. The framework would support improved generation, organization, and interpretation of data as well as modeling and prediction, not replacement of models. The field of toxicology has seen the benefits of wide use of one or more organizational frameworks (e.g., mode and mechanism of action, adverse outcome pathway). These frameworks influence how experiments are designed, data are collected, curated, stored and interpreted and ultimately how data are used in risk assessment. Exposure science is poised to similarly benefit from broader use of a parallel organizational framework, which Dr. von Göetz correctly points out, is currently used in the exposure modeling community. In our view, the concepts used so effectively in the exposure modeling community, expanded upon in the AEP framework, could see wider adoption by the field as a whole. The value of such a framework was recognized by the National Academy of Sciences.3 Replacement of models, databases, or any application with the AEP framework was not proposed in our article. The positive role broader more consistent use of such a framework might have in enabling and advancing “general activities such as data acquisition, organization…,” and exposure modeling was discussed

  10. Expanding Awareness of Docosahexaenoic Acid during Pregnancy

    Directory of Open Access Journals (Sweden)

    Barbara J. Meyer

    2013-04-01

    Full Text Available Pregnant women do not currently meet the consensus recommendation for docosahexaenoic acid (DHA (≥200 mg/day. Pregnant women in Australia are not receiving information on the importance of DHA during pregnancy. DHA pregnancy education materials were developed using current scientific literature, and tested for readability and design aesthetics. The study aimed to evaluate their usefulness, the desire for pregnant women to receive these materials and whether a larger separate study (using a control group is warranted to evaluate the influence the materials may have on increasing DHA consumption in pregnant women in Australia. Pregnant women (N = 118 were recruited at antenatal clinics at two NSW hospitals. Participants completed a 16-item questionnaire and DHA educational materials (pamphlet and shopping card were provided. Participants were contacted via phone two weeks later and completed the second questionnaire (25-item, N = 74. Statistics were conducted in SPSS and qualitative data were analysed to identify common themes. Ninety three percent of women found the materials useful, with the main reason being it expanded their knowledge of DHA food sources. Only 34% of women had received prior information on DHA, yet 68% said they would like to receive information. Due to the small sample size and lack of a control group, this small study cannot provide a cause and effect relationship between the materials and nutrition related behaviours or knowledge, however the results indicate a potential positive influence towards increased fish consumption and awareness of DHA containing foods. This suggests a larger study, with a control group is warranted to identify the impact such materials could have on Australian pregnant women.

  11. Exposing medical students to expanding populations

    Directory of Open Access Journals (Sweden)

    Lindenthal JJ

    2015-03-01

    Full Text Available JJ Lindenthal,1,2 JA DeLisa,3 GF Heinrich,4 WS Calderón Gerstein,5 1Department of Psychiatry, Institute for the Public Understanding of Health and Medicine, 2Department of Physical Medicine and Rehabilitation, New Jersey Medical School, Rutgers University, Newark, NJ, USA; 3Department of Orthopedics and Rehabilitation, University of New Mexico Health Science Center, Albuquerque, NM, USA; 4Department of Preventive Medicine and Community Health, New Jersey Medical School, Rutgers University, Newark, NJ, USA; 5Hospital Nacional Ramiro Prialé, EsSalud, Huancayo, Peru Abstract: Physicians are required to advocate for and counsel patients based on the best science and the interests of the individual while avoiding discrimination, ensuring equal access to health and mental services. Nonetheless, the communication gap between physician and patients has long been observed. To this end, the Institute for the Public Understanding of Health and Medicine of the Rutgers University New Jersey Medical School has expanded its efforts. This report describes two new programs: a legacy lecture series for medical students and an international “experience”, in Huancayo, Peru, for medical students and faculty. The MiniMed outreach program, now in its ninth year and first described in this journal in 2012, was designed to empower the powerless to communicate more effectively with clinicians, thus improving both the effectiveness of the physician–patient relationship and health care outcomes. The approach of the two new programs and their effects on patients, particularly the underserved, and medical students and faculty, are outlined in the following article. Keywords: MiniMed program, equal access, underserved populations, Newark Renaissance House, Kintock Group, role modeling 

  12. AstroCom NYC: Expanding the Partnership

    Science.gov (United States)

    Paglione, Timothy; Ford, Saavik; Agueros, Marcel A.; Mac Low, Mordecai-Mark; Robbins, Dennis

    2015-01-01

    AstroCom NYC is an undergraduate mentoring program designed to improve urban minority student access to opportunities in astrophysical research by greatly enhancing partnerships between research astronomers in New York City (City University of New York - an MSI, American Museum of Natural History, and Columbia). AstroCom NYC provides centralized, personalized mentoring as well as financial and academic support, to CUNY undergraduates throughout their studies, plus the resources and opportunities to further CUNY faculty research with students. The goal is that students' residency at AMNH helps them build a sense of belonging in the field, and readies and inspires them for graduate study. AstroCom NYC provides a rigorous Methods of Scientific Research course developed specifically to this purpose, a laptop, research and career mentors, outreach activities, scholarships and stipends, Metrocards, and regular assessment for maximum effectiveness. Stipends in part alleviate the burdens at home typical for CUNY students so they may concentrate on their academic success. AMNH serves as the central hub for our faculty and students, who are otherwise dispersed among all five boroughs of the City. For our second cohort, we dramatically improved the application and screening process, implemented a number of tools to evaluate their potential for grad school, and began growing a network of potential hosts for summer internships around NY State and the US. We review these implementations and outcomes, as well as plans for Year 3, when we expect many of our current students to compete for external summer REUs, and after greatly expanding the program reach through a NASA community college initiative.

  13. Expanding Lookout Capabilities for Architectural Analysis

    Science.gov (United States)

    Shick, B.

    SMC/SYSW/ENY's Lookout tool provides a M&S capability for architectural analysis. It models the contributions of ground and space-based assets in several mission threads and scenarios to quantify overall Space Situational Awareness (SSA) capability. Plotting performance results versus costs enables decision makers to identify and evaluate Best Value families of systems and combinations of architectures. Currently, SMC intends to use Lookout to impact the Fiscal Year 2012 budget programming cycle, the National SSA Initial Capabilities Document (ICD) and Architecture definition effort, planning for programs of record, and AFSPC & SMC leadership. Ultimately, Lookout will enable additional space superiority analysis. Previous Lookout work focused on modeling the metric tracking capabilities of the Space Surveillance Network (detecting and tracking) and proposed concepts to close identified collection shortfalls. SMC/SYSW/ENY leveraged some of the lessons learned in developing and implementing the metric tracking models to expand Lookout to develop an initial characterization capability, including non-resolved space object identification (SOI), imaging, and Foreign Instrumentation and Signals (FIS) Intelligence. Characterization collection phenomenologies added in FY08 and FY09 include mechanical tracking and phased array radars, visible telescopes, and signals collection. Lookout enables evaluating the characterization collections for quantity, quality, and timeliness. Capturing the Tasking, Collection, Processing, Exploitation, and Dissemination processes represent one of the biggest challenges in including characterization capabilities in mission thread and scenario-based analysis. The SMC/SYSW/ENY team met with several representatives of the community and held community-wide Technical Interchange Meetings. Based on feedback from these meetings, SMC created an infrastructure for modeling the tasking processes and scales to relate collection quality to intelligence

  14. Gonadotropin releasing hormone agonists: Expanding vistas

    Directory of Open Access Journals (Sweden)

    Navneet Magon

    2011-01-01

    Full Text Available Gonadotropin-releasing hormone (GnRH agonists are derived from native GnRH by amino acid substitution which yields the agonist resistant to degradation and increases its half-life. The hypogonadotropic hypogonadal state produced by GnRH agonists has been often dubbed as "pseudomenopause" or "medical oophorectomy," which are both misnomers. GnRH analogues (GnRH-a work by temporarily "switching off" the ovaries. Ovaries can be "switched off" for the therapy and therapeutic trial of many conditions which include but are not limited to subfertility, endometriosis, adenomyosis, uterine leiomyomas, precocious puberty, premenstrual dysphoric disorder, chronic pelvic pain, or the prevention of menstrual bleeding in special clinical situations. Rapidly expanding vistas of usage of GnRH agonists encompass use in sex reassignment of male to female transsexuals, management of final height in cases of congenital adrenal hyperplasia, and preserving ovarian function in women undergoing cytotoxic chemotherapy. Hypogonadic side effects caused by the use of GnRH agonists can be tackled with use of "add-back" therapy. Goserelin, leuprolide, and nafarelin are commonly used in clinical practice. GnRH-a have provided us a powerful therapeutic approach to the treatment of numerous conditions in reproductive medicine. Recent synthesis of GnRH antagonists with a better tolerability profile may open new avenues for both research and clinical applications. All stakeholders who are partners in women′s healthcare need to join hands to spread awareness so that these drugs can be used to realize their full potential.

  15. AIDSCAP initiative expands prevention efforts for women.

    Science.gov (United States)

    1995-01-01

    By 2000, women will likely comprise 50% of HIV-infected persons worldwide. Women, regardless of number of sexual partners, are especially vulnerable to HIV. One act of intercourse puts women at an 18 times greater risk than men of acquiring HIV. Traditional gender roles denying women the power to protect their own health exacerbate their biological susceptibility to HIV infection. AIDSCAP's Women's Initiative (AWI) aims to secure culturally and gender-sensitive AIDSCAP interventions and strategies and to advance AIDSCAP's leadership role in developing HIV/AIDS prevention models for women. More than 50% of AIDSCAP current projects target women. AWI integrates a broader gender perspective into AIDSCAP's project design, technical assistance, evaluation and monitoring, and policy projects. It has expanded target women's groups beyond the groups traditionally considered at high risk. To date, various AIDSCAP projects have trained and educated about one million women. The Christian Reformed World Relief Committee (CRWRC) is involved in HIV/AIDS prevention activities. Its AIDS and Sexually Transmitted Disease (STD) Health Promotion Prevention Program with Market Women in Senegal targets self-employed market women and urban female youth in Dakar, Kaoloack, and Thies. CRWRC worked with two women's groups to develop a sexual health promotion package with HIV/AIDS and STD IEC (information, education, and communication) materials and has trained 20 facilitators to use the promotion package. It helps the two collaborating groups to develop their organization capacities so they can implement and evaluate sustainable HIV/STD prevention programs. AIDSCAP also supports training of Kenyan family planning provider activities in HIV prevention of the JSI Family Planning Sector Project. It collaborates with the Center of Women Workers in Haiti to provide women factory workers IEC and condoms to promote risk reduction behavior. AWI also conducts research and policy activities (e

  16. The Solar Convection Spectrum

    Science.gov (United States)

    Bachmann, Kurt T.

    2000-01-01

    I helped to complete a research project with NASA scientists Dr. David Hathaway (my mentor), Rick Bogart, and John Beck from the SOHO/SOI collaboration. Our published paper in 'Solar Physics' was titled 'The Solar Convection Spectrum' (April 2000). Two of my undergraduate students were named on the paper--Gavrav Khutri and Josh Petitto. Gavrav also wrote a short paper for the National Conference of Undergraduate Research Proceedings in 1998 using a preliminary result. Our main result was that we show no evidence of a scale of convection named 'mesogranulation'. Instead, we see only direct evidence for the well-known scales of convection known as graduation and supergranulation. We are also completing work on vertical versus horizontal flow fluxes at the solar surface. I continue to work on phase relationships of solar activity indicators, but I have not yet written a paper with my students on this topic. Along with my research results, I have developed and augmented undergraduate courses at Birmingham-Southern College by myself and with other faculty. We have included new labs and observations, speakers from NASA and elsewhere, new subject material related to NASA and space science. I have done a great deal of work in outreach, mostly as President and other offices in the Birmingham Astronomical Society. My work includes speaking, attracting speakers, giving workshops, and governing.

  17. PINS Spectrum Identification Guide

    Energy Technology Data Exchange (ETDEWEB)

    A.J. Caffrey

    2012-03-01

    The Portable Isotopic Neutron Spectroscopy—PINS, for short—system identifies the chemicals inside munitions and containers without opening them, a decided safety advantage if the fill chemical is a hazardous substance like a chemical warfare agent or an explosive. The PINS Spectrum Identification Guide is intended as a reference for technical professionals responsible for the interpretation of PINS gamma-ray spectra. The guide is divided into two parts. The three chapters that constitute Part I cover the science and technology of PINS. Neutron activation analysis is the focus of Chapter 1. Chapter 2 explores PINS hardware, software, and related operational issues. Gamma-ray spectral analysis basics are introduced in Chapter 3. The six chapters of Part II cover the identification of PINS spectra in detail. Like the PINS decision tree logic, these chapters are organized by chemical element: phosphorus-based chemicals, chlorine-based chemicals, etc. These descriptions of hazardous, toxic, and/or explosive chemicals conclude with a chapter on the identification of the inert chemicals, e.g. sand, used to fill practice munitions.

  18. Rotational Spectrum of Saccharine

    Science.gov (United States)

    Alonso, Elena R.; Mata, Santiago; Alonso, José L.

    2017-06-01

    A significant step forward in the structure-activity relationships of sweeteners was the assignment of the AH-B moiety in sweeteners by Shallenberger and Acree. They proposed that all sweeteners contain an AH-B moiety, known as glucophore, in which A and B are electronegative atoms separated by a distance between 2.5 to 4 Å. H is a hydrogen atom attached to one of the electronegative atom by a covalent bond. For saccharine, one of the oldest artificial sweeteners widely used in food and drinks, two possible B moieties exist ,the carbonyl oxygen atom and the sulfoxide oxygen atom although there is a consensus of opinion among scientists over the assignment of AH-B moieties to HN-SO. In the present work, the solid of saccharine (m.p. 220°C) has been vaporized by laser ablation (LA) and its rotational spectrum has been analyzed by broadband CP-FTMW and narrowband MB-FTMW Fourier transform microwave techniques. The detailed structural information extracted from the rotational constants and ^{14}N nuclear quadrupole coupling constants provided enough information to ascribe the glucophore's AH and B sites of saccharine. R. S. Shallenberger, T. E. Acree. Nature 216, 480-482 Nov 1967. R. S. Shallenberger. Taste Chemistry; Blackie Academic & Professional, London, (1993).

  19. The Cu II Spectrum

    Directory of Open Access Journals (Sweden)

    Alexander Kramida

    2017-02-01

    Full Text Available New wavelength measurements in the vacuum ultraviolet (VUV, ultraviolet and visible spectral regions have been combined with available literature data to refine and extend the description of the spectrum of singly ionized copper (Cu II. In the VUV region, we measured 401 lines using a concave grating spectrograph and photographic plates. In the UV and visible regions, we measured 276 lines using a Fourier-transform spectrometer. These new measurements were combined with previously unpublished data from the thesis of Ross, with accurate VUV grating measurements of Kaufman and Ward, and with less accurate older measurements of Shenstone to construct a comprehensive list of ≈2440 observed lines, from which we derived a revised set of 379 optimized energy levels, complemented with 89 additional levels obtained using series formulas. Among the 379 experimental levels, 29 are new. Intensities of all lines observed in different experiments have been reduced to the same uniform scale by using newly calculated transition probabilities (A-values. We combined our calculations with published measured and calculated A-values to provide a set of 555 critically evaluated transition probabilities with estimated uncertainties, 162 of which are less than 20%.

  20. The worldwide leaf economics spectrum

    NARCIS (Netherlands)

    Wright, I.J.; Reich, P.B.; Westoby, M.; Ackerly, D.D.; Baruch, Z.; Bongers, F.J.J.M.; Cavender-Bares, J.; Chapin, T.; Cornelissen, J.H.C.; Diemer, M.; Flexas, J.; Garnier, E.; Groom, P.K.; Gulias, J.; Hikosaka, K.; Lamont, B.B.; Lee, T.; Lee, W.; Lusk, C.; Midgley, J.J.; Navas, M.L.; Niinements, Ü.; Oleksyn, J.; Osada, N.; Poorter, H.; Poot, P.; Prior, L.; Pyankov, V.I.; Roumet, C.; Thomas, S.C.; Tjoelker, M.G.; Veneklaas, E.J.; Villar, R.

    2004-01-01

    Bringing together leaf trait data spanning 2,548 species and 175 sites we describe, for the first time at global scale, a universal spectrum of leaf economics consisting of key chemical, structural and physiological properties. The spectrum runs from quick to slow return on investments of nutrients

  1. The spectrum of spinning superparticles

    NARCIS (Netherlands)

    Bergshoeff, E.; Holten, J.W. van

    1989-01-01

    We construct the action and transformation rules of a massless spinning superparticle in D dimensions which has N worldline as well as M target space supersymmetries. The spectrum of physical states for different values of N, M and D is presented. We also discuss the action, symmetries and spectrum

  2. Dynamic Spectrum Sharing among Femtocells

    DEFF Research Database (Denmark)

    Da Costa, Gustavo Wagner Oliveira

    2012-01-01

    The ever-growing demand for mobile broadband is leading to an imminent spectrum scarcity. In order to cope with such situation dynamic spectrum sharing and the widespread deployment of small cells (femtocells) are promising solutions. Delivering such a view is not short of challenges. Massive dep...

  3. Expander Families and Cayley Graphs A Beginner's Guide

    CERN Document Server

    Krebs, Mike

    2011-01-01

    The theory of expander graphs is a rapidly developing topic in mathematics and computer science, with applications to communication networks, error-correcting codes, cryptography, complexity theory, and much more. Expander Families and Cayley Graphs: A Beginner's Guide is a comprehensive introduction to expander graphs, designed to act as a bridge between classroom study and active research in the field of expanders. It equips those with little or no prior knowledge with the skills necessary to both comprehend current research articles and begin their own research. Central to this book are fou

  4. Infrastructure Requirements for an Expanded Fuel Ethanol Industry

    Energy Technology Data Exchange (ETDEWEB)

    Reynolds, Robert E. [Downstream Alternatives, Inc., South Bend, IN (United States)

    2002-01-15

    This report provides technical information specifically related to ethanol transportation, distribution, and marketing issues. This report required analysis of the infrastructure requirements for an expanded ethanol industry.

  5. Comparison of RF spectrum prediction methods for dynamic spectrum access

    Science.gov (United States)

    Kovarskiy, Jacob A.; Martone, Anthony F.; Gallagher, Kyle A.; Sherbondy, Kelly D.; Narayanan, Ram M.

    2017-05-01

    Dynamic spectrum access (DSA) refers to the adaptive utilization of today's busy electromagnetic spectrum. Cognitive radio/radar technologies require DSA to intelligently transmit and receive information in changing environments. Predicting radio frequency (RF) activity reduces sensing time and energy consumption for identifying usable spectrum. Typical spectrum prediction methods involve modeling spectral statistics with Hidden Markov Models (HMM) or various neural network structures. HMMs describe the time-varying state probabilities of Markov processes as a dynamic Bayesian network. Neural Networks model biological brain neuron connections to perform a wide range of complex and often non-linear computations. This work compares HMM, Multilayer Perceptron (MLP), and Recurrent Neural Network (RNN) algorithms and their ability to perform RF channel state prediction. Monte Carlo simulations on both measured and simulated spectrum data evaluate the performance of these algorithms. Generalizing spectrum occupancy as an alternating renewal process allows Poisson random variables to generate simulated data while energy detection determines the occupancy state of measured RF spectrum data for testing. The results suggest that neural networks achieve better prediction accuracy and prove more adaptable to changing spectral statistics than HMMs given sufficient training data.

  6. In vitro comparison of self-expanding versus balloon-expandable stents in a human ex vivo model.

    Science.gov (United States)

    Grenacher, Lars; Rohde, Stefan; Gänger, Ellen; Deutsch, Jochen; Kauffmann, Günter W; Richter, Götz M

    2006-01-01

    The objective was to compare the radial strength and expansile precision of self-expanding stents and balloon-expandable stents in a human cadaver bifurcation model. Seven different self-expanding (LUMINEXX, JOSTENT SelfX, JOSTENT SelfX hrf, Sinus-Repo, Sinus SuperFlex, Easy Wallstent, SMART) and four different balloon-expandable stent models (Palmaz, Sinus Stent, SAXX Medium, JOSTENT peripheral), each type 10 stents (total n = 110 stents) were implanted into the common iliac arteries of human cadaver corpses. The maximum stent diameter was 10 mm for all models. After stent implantation, the specimens were filled with silicone caoutchouc. After 24 h, the vascular walls including the stents were removed from the hardened casts. Diameters were taken and the weight of the cast cylinders was measured in air and in purified water to calculate the volume of the bodies (according to Archimedes Law) as a relative but precise degree for the radial strength of the implanted stents. The cylindrical casts of the self-expanding stents showed lower mean diameters (8.2 +/- 1.0 mm) and mean volumes (0.60 +/- 0.14 ml/cm) than in the balloon-expandable stent group (10.1 +/- 0.3 mm and 0.71 +/- 0.04 ml/cm, respectively; p < 0.01). The nominal maximum diameter of 10 mm was not achieved in any of the self-expanding stents, but this was achieved in more than 70% (29/40) of the balloon-expandable stent specimens (p < 0.05). The variation between achieved volumes was significantly larger in self-expanding (range: 0.23-0.78 ml/cm) than in balloon-expandable stents (range: 0.66-0.81 ml/cm; p < 0.05). Self-expanding stents presented considerably lower radial expansion force and lower degree of precision than balloon-expandable stents.

  7. Expanding policy options for educating teenagers.

    Science.gov (United States)

    Stern, David

    2009-01-01

    David Stern argues that some basic features of the American high school must be modified if it is to serve all students successfully. He notes, for example, that only three-quarters of U.S. high school students graduate four years after beginning ninth grade and that the National Assessment of Educational Progress found no improvement in reading or mathematics for seventeen-year-olds between 1971 and 2004. The nation's system for educating teenagers, says Stern, seems to be stuck, despite the constant efforts of teachers and repeated waves of reform. Citing two widely accepted public purposes of educating teenagers-preparation for civic participation and for economic self-sufficiency-Stern proposes four new strategies to achieve those goals. He draws on empirical evidence suggesting that these are promising directions for research and policy, but acknowledges that existing studies provide only limited guidance. First, he says, schools should continue the current trend toward integrating educational options to provide young people with skills and experiences that pave the way to both college and careers. Second, states and districts should tie education funding not simply to the number of students attending school, but also to what young people learn, whether they graduate, and whether they find jobs or enroll in postsecondary education. Such a move, he argues, would encourage teaching and learning formats that use students' time more effectively. Third, more adults in addition to classroom teachers should be involved in educating teenagers. Other adults acting as academic advisers, learning coaches, student advocates, internship supervisors, mentors, and college counselors could help guide the education of teenagers inside and outside of school and provide some relief for the chronic shortage of teachers. Fourth, schools should expand the options for educating teenagers outside of geographically fixed schools. Combining improved Internet-based curriculum with

  8. True CMB Power Spectrum Estimation

    CERN Document Server

    Paykari, P; Fadili, M J

    2012-01-01

    The cosmic microwave background (CMB) power spectrum is a powerful cosmological probe as it entails almost all the statistical information of the CMB perturbations. Having access to only one sky, the CMB power spectrum measured by our experiments is only a realization of the true underlying angular power spectrum. In this paper we aim to recover the true underlying CMB power spectrum from the one realization that we have without a need to know the cosmological parameters. The sparsity of the CMB power spectrum is first investigated in two dictionaries; Discrete Cosine Transform (DCT) and Wavelet Transform (WT). The CMB power spectrum can be recovered with only a few percentage of the coefficients in both of these dictionaries and hence is very compressible in these dictionaries. We study the performance of these dictionaries in smoothing a set of simulated power spectra. Based on this, we develop a technique that estimates the true underlying CMB power spectrum from data, i.e. without a need to know the cosmo...

  9. An Expanding Locally Anisotropic (ELA) Metric Describing Matter in an Expanding Universe

    CERN Document Server

    Ferreira, P Castelo

    2010-01-01

    It is suggested an expanding locally anisotropic metric (ELA) ansatz describing matter in a flat expanding universe which interpolates between the Schwarzschild (SC) metric near point-like central bodies of mass 'M' and the Robertson-Walker (RW) metric for large radial coordinate: 'ds^2=Z(cdt)2 - 1/Z (dr1-(Hr1/c) Z^(alpha/2+1/2)(cdt))^2-r1^2 dOmega', where 'Z=1-U' with 'U=2GM/(c^2r1)', 'G' is the Newton constant, 'c' is the speed of light, 'H=H(t)=\\dot(a)/a' is the time-dependent Hubble rate, 'dOmega=dtheta^2+sin^2(theta) dvarphi^2' is the solid angle element, 'a' is the universe scale factor and we are employing the coordinates 'r1=ar', being 'r' the radial coordinate for which the RW metric is diagonal. For constant exponent 'alpha=alpha0=0' it is retrieved the isotropic McVittie (McV) metric and for 'alpha=alpha0=1' it is retrieved the locally anisotropic Cosmological-Schwarzschild (SCS) metric, both already discussed in the literature. However it is shown that only for constant exponent 'alpha=alpha0> 1' ...

  10. An Isothermal Steam Expander for an Industrial Steam Supplying System

    Directory of Open Access Journals (Sweden)

    Chen-Kuang Lin

    2015-01-01

    Full Text Available Steam is an essential medium used in the industrial process. To ensure steam quality, small and middle scale boilers are often adopted. However, because a higher steam pressure (compared to the necessary steam pressure is generated, the boiler’s steam pressure will be reduced via a pressure regulator before the steam is directed through the process. Unfortunately, pressure is somewhat wasted during the reducing process. Therefore, in order to promote energy efficiency, a pressure regulator is replaced by a steam expander. With this steam expander, the pressure will be transformed into mechanical energy and extracted during the expansion process. A new type of isothermal steam expander for an industrial steam supplying system will be presented in the paper. The isothermal steam expander will improve the energy efficiency of a traditional steam expander by replacing the isentropic process with an isothermal expansion process. With this, steam condensation will decrease, energy will increase, and steam quality will be improved. Moreover, the mathematical model of the isothermal steam expander will be established by using the Schmidt theory, the same principle used to analyze Stirling engines. Consequently, by verifying the correctness of the theoretical model for the isothermal steam expander using experimental data, a prototype of 100 c.c. isothermal steam expander is constructed.

  11. Principles of the fifth order tuning of beam expanders

    Energy Technology Data Exchange (ETDEWEB)

    Meot, F.; Aniel, T.

    1995-12-31

    An analytical treatment of the third and fifth order optics of beam expanders is described, which allows precise tuning of the optical elements of the beam line, and efficient optimization of the beam uniformizing at the extended target. An application to a two-dimensional expander is given as an illustration. (authors). 4 refs.

  12. 47 CFR 69.121 - Connection charges for expanded interconnection.

    Science.gov (United States)

    2010-10-01

    ... 47 Telecommunication 3 2010-10-01 2010-10-01 false Connection charges for expanded interconnection... interconnection. (a) Appropriate connection charge subelements shall be established for the use of equipment and facilities that are associated with offerings of expanded interconnection for special access and...

  13. Expanded Polystyrene Re-Expansion Analysis Following Impact Compression

    Science.gov (United States)

    2015-03-04

    USAARL Report No. 2015-08 Expanded Polystyrene Re-Expansion Analysis Following Impact Compression By Mark S. Adams Frederick Brozoski Katie...13 iv This page is intentionally left blank. 1 Introduction Expanded bead polystyrene (EPS) is widely...EPS energy attenuating liners typically have complex geometric shapes. However, the use of flat sheets of polystyrene facilitated the sample

  14. Gamma spectrum following neutron capture in {sup 167}Er

    Energy Technology Data Exchange (ETDEWEB)

    Visser, D.; Khoo, T.L.; Lister, C.J. [and others

    1995-08-01

    Statistical decay from a highly excited state samples all the lower-lying states and, hence, provides a sensitive measure of the level density. Pairing has a major impact on the level density, e.g. creating a pair gap between the 0- and 2-quasiparticle configurations. Hence the shape of the statistical spectrum contains information on pairing, and can be used to provide information on the reduction of pairing with thermal excitation energy. For this reason, we measured the complete spectrum of {gamma}rays following thermal neutron capture in {sup 167}Er. The experiment was performed at the Brookhaven reactor using Compton-suppressed Ge detectors from TESSA. The spectrum, which was corrected for detector response and efficiency, reveals primary (first-step, high-energy) transitions up to nearly 8 MeV, secondary (last-step, lower-energy) transitions, as we as a continuous statistical component. Effort was expanded to identify all lines from contaminant sources and an upper limit of 5% was tentatively set for their contributions. The spectral shape of the statistical spectrum will be compared with theoretical spectra obtained from a calculation of pairing which accounts for a stepwise reduction of the pair correlations as the number of quasiparticles increases. The primary lines which decay directly to the near-yrast states will also be used to deduce the level densities.

  15. SPECTRUM OF OPPORTUNISTIC INFECTIONS IN HIV-AIDS PATIENTS

    Directory of Open Access Journals (Sweden)

    S S Madkar

    2012-10-01

    Full Text Available Aim: AIDS is characterized by a number of opportunistic infections which are responsible for high morbidity and mortality. The spectrum and distribution of opportunistic infections (OIs in AIDS patients is ever-expanding. This spectrum varies from continent to continent. The aim of the present study was to document the spectrum of OIs in HIV-infected patients in Ambajogai. Material and Method: 178 HIV positive symptomatic patients, either hospitalized or coming to ART (Antiretroviral Therapy centre in S.R.T.R. Medical College, Ambajogai, were included in the study for finding the spectrum of opportunistic infections. Result: The commonest opportunistic infection seen was tuberculosis (59% of patients, followed by oral candidiasis (37.6% of patients and parasitic diarrhea due to Cryptosporidium parvum(18 % of patients. It was observed that out of 178 patients, maximum 53.3% were in the age group of 29-38 years followed by 21% in the age group of 39-48 years. It was found that 77% were males and 23% were females, with male to female ratio is 3.3:1. Conclusion: This study demonstrates that tuberculosis is the commonest opportunistic infection seen in HIV patients. Clinicians should consider HIV in the differential diagnosis and management of all persons with tuberculosis.

  16. The spectrum of Cosmic Rays escaping from relativistic shocks

    CERN Document Server

    Katz, Boaz; Waxman, Eli

    2010-01-01

    We derive expressions for the time integrated spectrum of Cosmic Rays (CRs) that are accelerated in a decelerating relativistic shock wave and escape ahead of the shock. It is assumed that at any given time the CRs have a power law form, carry a constant fraction of the energy E_tot of the shocked plasma, and escape continuously at the maximal energy attainable. The spectrum of escaping particles is highly sensitive to the instantaneous spectral index due to the fact that the minimal energy, E_min ~ \\Gamma^2 m_pc^2 where \\Gamma is the shock Lorentz factor, changes with time. In particular, the escaping spectrum may be considerably harder than the canonical N(E)\\propto E^-2 spectrum. For a shock expanding into a plasma of density n, a spectral break is expected at the maximal energy attainable at the transition to non relativistic velocities, E ~ 10^19 (\\epsilon_B/0.1)(n/1 cm^-3)^(1/6)(E_tot/10^51 erg)^(1/3) eV where \\epsilon_B is the fraction of the energy flux carried by the magnetic field. If ultra-high ene...

  17. Fundamentals of spread spectrum modulation

    CERN Document Server

    Ziemer, Rodger E

    2007-01-01

    This lecture covers the fundamentals of spread spectrum modulation, which can be defined as any modulation technique that requires a transmission bandwidth much greater than the modulating signal bandwidth, independently of the bandwidth of the modulating signal. After reviewing basic digital modulation techniques, the principal forms of spread spectrum modulation are described. One of the most important components of a spread spectrum system is the spreading code, and several types and their characteristics are described. The most essential operation required at the receiver in a spread spect

  18. Broad-spectrum antiviral agents

    Directory of Open Access Journals (Sweden)

    Jun-Da eZhu

    2015-05-01

    Full Text Available Development of highly effective, broad-spectrum antiviral agents is the major objective shared by the fields of virology and pharmaceutics. Antiviral drug development has focused on targeting viral entry and replication, as well as modulating cellular defense system. High throughput screening of molecules, genetic engineering of peptides, and functional screening of agents have identified promising candidates for development of optimal broad-spectrum antiviral agents to intervene in viral infection and control viral epidemics. This review discusses current knowledge, prospective applications, opportunities, and challenges in the development of broad-spectrum antiviral agents.

  19. AUTISM SPECTRUM DISORDERS (ASD

    Directory of Open Access Journals (Sweden)

    Middha Akanksha

    2011-05-01

    Full Text Available Autism or Autism Spectrum Disorders (ASD is a serious neurological disorder affecting communication skills, social interactions, adaptability in an individual, and also causes dramatic changes in behavioral patterns. This condition typically lasts throughout one’s lifetime and affects both, children as well as adults. Research has shown a tenfold increase in autism cases over the past decade and still rising at an alarming pace. The origins of autism are not known even to modern science. Autism exists at different levels in individuals affected by the disease and is classified into five types. Symptoms for autism are more pronounced and prevalent in children compared to adults. Though some studies attribute autism to gene abnormality, science is yet to furnish hard facts about exact autism causes. Scientists and doctors are also unanimous in their opinion that autism, as of yet, has no cure. Treatments of autism are widely available and help in alleviating the symptoms of autism which make living with the condition easier.Several factors work together in causing autism but isolation and identification of a chief cause or causes has yet to be accomplished by modern science. Some people mistakenly believe that autism is related to bad parenting, vaccinations, or malnutrition. But these misconceptions are due to improper knowledge related to the disease. Symptoms of autism usually surface within the first two years of birth in children. Autistic children usually avoid eye contact and are poor imitators of sound together with a disliking towards a change in routines as well as non adaptability to new environments. At present, there is an absence of medical tests which can diagnose autism. The diagnosis of autism is largely based on developmental history and behavioral patterns. Medicinal treatments of autism have a downside as autism patients develop resistance to certain drugs over long period of use. All types of autism demand a good plan of

  20. Cyclostationary Beacon for Assisting Spectrum Sensing in Opportunistic Spectrum Access

    Directory of Open Access Journals (Sweden)

    Thomas Kaiser

    2011-03-01

    Full Text Available Cognitive radio is a promising solution to the problem of spectrum scarcity by means of allowing secondary radio networks access the spectrum opportunistically. One of the most important issues in cognitive radio is how to detect existing over-the-air signals reliably. Not a few literatures have reported that signals could be detected via their inherent or embedded properties. However, this approach may not be reliable and flexible enough for all kinds of signals with different modulation types. In this paper, we propose a type of multitone beacon signal carrying cyclostationary signatures, which is able to enhance the reliability and efficiency of signal detection at low cost of spectrum overhead. This beacon not only can indicate the presence or absence of user signal but also can reveal some other information helpful to opportunistic spectrum access through the information bits carried on its cyclostationary signatures. It could be applied to device/network identification, indication of spectrum allocation and spectrum rendezvous, both for primary and secondary users. Based on our previous work reported in [1], the generation and detection algorithm of the beacon signal are extended with improved spectral efficiency. Performance is discussed with both computer simulation and testbed validation.

  1. The helical screw expander evaluation project. [for geothermal wells

    Science.gov (United States)

    Mckay, R. A.

    1977-01-01

    A positive-displacement helical-screw expander of the Lysholm type has been adapted for geothermal service and successfully demonstrated in a 50 kW prototype power system. Evaluation of the expander by tests of a new model in a 1 MW power system under wellhead conditions in selected liquid-dominated geothermal fields is proposed. The objectives are to determine the performance characteristics of the expander and power system over a broad range of operating conditions and also to examine the concept of wellhead power plants. Throttling and fractionation of the fluids from the test wells is planned to simulate a wide range of wellhead pressures and steam fractions. Variation in the expander exhaust pressure is also planned. The investigation will include expander efficiency, corrosion, erosion, scale formation and control, and endurance testing. Interaction studies with the wells and an electric grid are also proposed.

  2. Autism Spectrum Disorder and Fragile X Syndrome

    Science.gov (United States)

    ... Just Figuring Out CGG Repeats! Donate Print PDF Autism Spectrum Disorder and Fragile X Syndrome Fragile X ... known single gene cause of ASD What Is Autism Spectrum Disorder? Read my Story Autism spectrum disorder ( ...

  3. Autism Spectrum Disorder and Fragile X Syndrome

    Science.gov (United States)

    ... only after another family member has been diagnosed. Autism Spectrum Disorder and Fragile X Syndrome Fragile X ... known single gene cause of ASD What Is Autism Spectrum Disorder? Autism spectrum disorder (ASD) is a ...

  4. Longitudinal peak detected Schottky spectrum

    CERN Document Server

    Shaposhnikova, E

    2009-01-01

    The "peak detected Schottky" spectrum is a diagnostic used since the late seventies for beam observation in the SPS and now already applied to the LHC. This tool was always believed, however without proof, to give a good picture of the particle distribution in synchrotron frequencies similar to the longitudinal Schottky spectrum of unbunched beam for revolution frequencies.In this paper an analysis of this measurement technique is presented both in a general form and for the particular realisation in the SPS. In addition the limitations of the present experimental set-up are discussed together with possible improvements. The analysis shows that for an optimised experimental set-up the spectrum of the peak detected signal is very close to the synchrotron frequency distribution inside the bunch - much closer than that given by the traditional longitudinal bunched-beam Schottky spectrum.

  5. Spectrum Trading in India and 5G

    DEFF Research Database (Denmark)

    Tripathi, Purnendu; Prasad, Ramjee

    2013-01-01

    Currently radio spectrum is largely managed through Command and Control method. Public mobile services require spectrum below 3 GHz for providing cost effective services. The existing method has created artificial shortage of spectrum especially below 3 GHz. Spectrum trading is a new concept in w...... for spectrum trading and it could provide a boost to the Indian telecom sector. Further, it will also discuss spectrum issue related with 5G in the direction of millimeter waves....

  6. Beyond the Spectrum: Rethinking Autism

    Directory of Open Access Journals (Sweden)

    Heather Thomas

    2017-03-01

    Full Text Available The "spectrum" has become the dominant metaphor for conceptualizing autism, with fundamental consequences for notions of disability, diversity, and normality. In this article, we draw on ethnographic research with autistic communities to explore how the notion of the autism spectrum has become a focus of explicit identification, reflection, and contestation. To further this inquiry, we place these debates into conversation with earlier debates regarding another spectrum—the Kinsey Scale, a "spectrum" for conceptualizing sexual orientation that first appeared in 1948 but has been critiqued since the 1970s. How might responses to the Kinsey Scale (like the Klein Grid contribute to rethinking the autism spectrum? This is a question about the cultural and political implications of metaphors and conceptual models. It is of broad importance because the spectrum metaphor is being extended to a range of conditions beyond autism itself. Our goal is thus to build on insights from sexuality studies as well as the insights of autistic persons, advocates, and researchers who wish to forestall the naturalization of "the spectrum." In doing so, we seek to contribute to a discussion of what alternative frameworks might bring to questions of social justice, ability, and human flourishing.

  7. Microbiome Disturbances and Autism Spectrum Disorders.

    Science.gov (United States)

    Rosenfeld, Cheryl S

    2015-10-01

    Autism spectrum disorders (ASDs) are considered a heterogenous set of neurobehavioral diseases, with the rates of diagnosis dramatically increasing in the past few decades. As genetics alone does not explain the underlying cause in many cases, attention has turned to environmental factors as potential etiological agents. Gastrointestinal disorders are a common comorbidity in ASD patients. It was thus hypothesized that a gut-brain link may account for some autistic cases. With the characterization of the human microbiome, this concept has been expanded to include the microbiota-gut-brain axis. There are mounting reports in animal models and human epidemiologic studies linking disruptive alterations in the gut microbiota or dysbiosis and ASD symptomology. In this review, we will explore the current evidence that gut dysbiosis in animal models and ASD patients correlates with disease risk and severity. The studies to date have surveyed how gut microbiome changes may affect these neurobehavioral disorders. However, we harbor other microbiomes in the body that might impact brain function. We will consider microbial colonies residing in the oral cavity, vagina, and the most recently discovered one in the placenta. Based on the premise that gut microbiota alterations may be causative agents in ASD, several therapeutic options have been tested, such as diet modulations, prebiotics, probiotics, synbiotics, postbiotics, antibiotics, fecal transplantation, and activated charcoal. The potential benefits of these therapies will be considered. Finally, the possible mechanisms by which changes in the gut bacterial communities may result in ASD and related neurobehavioral disorders will be examined.

  8. Tissue regeneration during tissue expansion and choosing an expander

    Directory of Open Access Journals (Sweden)

    K Agrawal

    2012-01-01

    Full Text Available This paper reviews the various aspects of tissue regeneration during the process of tissue expansion. "Creep" and mechanical and biological "stretch" are responsible for expansion. During expansion, the epidermis thickens, the dermis thins out, vascularity improves, significant angiogenesis occurs, hair telogen phase becomes shorter and the peripheral nerves, vessels and muscle fibres lengthen. Expansion is associated with molecular changes in the tissue. Almost all these biological changes are reversible after the removal of the expander.This study is also aimed at reviewing the difficulty in deciding the volume and dimension of the expander for a defect. Basic mathematical formulae and the computer programmes for calculating the dimension of tissue expanders, although available in the literature, are not popular. A user-friendly computer programme based on the easily available Microsoft Excel spread sheet has been introduced. When we feed the area of defect and base dimension of the donor area or tissue expander, this programme calculates the volume and height of the expander. The shape of the expander is decided clinically based on the availability of the donor area and the designing of the future tissue movement. Today, tissue expansion is better understood biologically and mechanically. Clinical judgement remains indispensable in choosing the size and shape of the tissue expander.

  9. Novel pricing model for spectrum leasing in secondary spectrum market

    Institute of Scientific and Technical Information of China (English)

    WANG Lei; XU Xing-kun; XU Wen-jun; HE Zhi-qiang; LIN Jia-ru

    2010-01-01

    According to the property rights model of cognitive radio,primary users who own the spectral resource have the right to lease or trade part of it to secondary users in exchange for appropriate profit. In this paper,an implementation of this framework is investigated,where a primary link can lease the owned spectrum to secondary nodes in exchange for cooperation (relaying). A novel pricing model is proposed that enables the trading between spectrum and cooperation. Based on the demand of secondary nodes,the primary link attempts to maximize its quality of service (QoS) by setting the price of spectrum. Taking the price asked by primary link,the secondary nodes aim to obtain most profits by deciding the amount of spectrum to buy and then pay for it by cooperative transmission. The investigated model is conveniently cast in the framework of seller/buyer (Stackelberg) games. Analysis and numerical results show that our pricing model is effective and practical for spectrum leasing based on trading spectral resource for cooperation.

  10. Balloon-expandable Metallic Stents for Airway Diseases.

    Science.gov (United States)

    Ohki, Takashi; Sugimoto, Seiichiro; Kurosaki, Takeshi; Otani, Shinji; Miyoshi, Kentaroh; Yamane, Masaomi; Miyoshi, Shinichiro; Oto, Takahiro

    2016-10-01

    Stent placement is an essential treatment for airway diseases. Although self-expandable metallic stents and silicone stents are commonly applied for the treatment of airway diseases, these stents are unsuitable for the treatment of small airway diseases encountered in pediatric patients and lung transplant recipients with airway complications. Currently, only vascular balloon-expandable metallic stents are available for the treatment of small airway diseases; however, little research has been conducted on the use of these stents in this field. We have launched a prospective feasibility study to clarify the safety and efficacy of balloon-expandable metallic stents for the treatment of airway diseases.

  11. Acute expanded perlite exposure with persistent reactive airway dysfunction syndrome.

    Science.gov (United States)

    Du, Chung-Li; Wang, Jung-Der; Chu, Po-Chin; Guo, Yue-Liang Leon

    2010-01-01

    Expanded perlite has been assumed as simple nuisance, however during an accidental spill out in Taiwan, among 24 exposed workers followed for more than 6 months, three developed persisted respiratory symptoms and positive provocation tests were compatible with reactive airway dysfunction syndrome. During simulation experiment expanded perlite is shown to be very dusty and greatly exceed current exposure permission level. Review of literature and evidence, though exposure of expanded perlite below permission level may be generally safe, precautionary protection of short term heavy exposure is warranted.

  12. Rupture of an expander prosthesis mimics axillary cancer recurrence.

    LENUS (Irish Health Repository)

    Ismael, T

    2005-10-01

    Regional silicone gel migration from a ruptured breast implant has been reported at different locations including the upper extremity, chest wall muscles, axilla and back. We report a patient who presented with an axillary mass that mimicked a regional recurrence 5 years after breast cancer reconstruction with a latissimus dorsi musculocutaneous flap and silicon gel expander-prosthesis. Surgical exploration revealed that the mass contained silicone gel around the port of the breast expander that had ruptured. The mass was confluent with an intracapsular silicone leak through a tract along the tube of the expander port.

  13. On The Relativity of Redshifts: Does Space Really "Expand"?

    CERN Document Server

    Lewis, Geraint F

    2016-01-01

    In classes on cosmology, students are often told that photons stretch as space expands, but just how physical is this picture? Does space really expand? In this article, we explore the notion of the redshift of light with Einstein's general theory of relativity, showing that the core underpinning principles reveal that redshifts are both simpler and more complex than you might naively think. This has significant implications for the observed redshifting of photons as they travel across the universe, often refereed to as the cosmological redshift, and for the idea of expanding space.

  14. The Spectrum and Laplacian Spectrum of the Dice Lattice

    Science.gov (United States)

    Li, Shuli; Yan, Weigen; Tian, Tao

    2016-07-01

    The dice lattice is the dual lattice of kagomé lattice. Many physical properties on the dice lattice have been studied by physicists, such as Ising model, Glassy dynamics of Josephson arrays, and Lattice Green's function. In this paper, we derive the spectrum and Laplacian spectrum of the dice lattice with toroidal boundary condition. In addition, we apply our results to obtain the formulae of the number of spanning trees, the Kirchhoff index, and the energy of the dice lattice with toroidal boundary condition.

  15. Expandable Cryogenic Tankage for On Orbit Depot Storage Project

    Data.gov (United States)

    National Aeronautics and Space Administration — The proposed expandable depot storage tanks would make it possible to have an on-orbit tank larger in volume than any existing or planned launch vehicles. The size...

  16. The Canonical Expanding Soliton and Harnack inequalities for Ricci flow

    CERN Document Server

    Cabezas-Rivas, Esther

    2009-01-01

    We introduce the notion of Canonical Expanding Ricci Soliton, and use it to derive new Harnack inequalities for Ricci flow. This viewpoint also gives geometric insight into the existing Harnack inequalities of Hamilton and Brendle.

  17. Overcoming the Barriers to Expanded Pharmacy Service--Pharmaceutical Education.

    Science.gov (United States)

    Campbell, William H.

    1979-01-01

    Cognitive, situational, and attitudinal barriers to expanding the role of the pharmacist in the health care services are discussed. Attention is focused on how pharmacy education can overcome some of these barriers. (SF)

  18. Massive expanding hematoma of the chin following blunt trauma

    Directory of Open Access Journals (Sweden)

    K Thanvir Mohamed Niazi

    2016-01-01

    Full Text Available Posttraumatic hematoma of the face is common and usually self-limiting in nature. We report an unusual massive expanding hematoma of the chin within 9 h following a blunt trauma with no associated injuries or fracture.

  19. Massive expanding hematoma of the chin following blunt trauma.

    Science.gov (United States)

    Niazi, K Thanvir Mohamed; Raja, Dharmesh Kumar; Prakash, R; Balaji, V R; Manikandan, D; Ulaganathan, G; Yoganandha, R

    2016-10-01

    Posttraumatic hematoma of the face is common and usually self-limiting in nature. We report an unusual massive expanding hematoma of the chin within 9 h following a blunt trauma with no associated injuries or fracture.

  20. Environmental assessment, expanded Ponnequin wind energy project, Weld County, Colorado

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1999-02-01

    The US Department of Energy (DOE) has considered a proposal from the State of Colorado, Office of Energy Conservation (OEC), for funding construction of the Expanded Ponnequin Wind Project in Weld County, Colorado. OEC plans to enter into a contracting arrangement with Public Service Company of Colorado (PSCo) for the completion of these activities. PSCo, along with its subcontractors and business partners, are jointly developing the Expanded Ponnequin Wind Project. The purpose of this Final Environmental Assessment (EA) is to provide DOE and the public with information on potential environmental impacts associated with the Expanded Ponnequin Wind Energy Project. This EA, and public comments received on it, were used in DOE`s deliberations on whether to release funding for the expanded project under the Commercialization Ventures Program.

  1. Outreach Plan : Sherburne National Wildlife Refuge for Expanded Hunting Opportunities

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — This Outreach Plan for Sherburne NWR states that hunting opportunities will be expanded on the Refuge to include wild turkey hunting by disabled and youth hunters.

  2. The Biokinetic Spectrum for Temperature.

    Directory of Open Access Journals (Sweden)

    Ross Corkrey

    Full Text Available We identify and describe the distribution of temperature-dependent specific growth rates for life on Earth, which we term the biokinetic spectrum for temperature. The spectrum has the potential to provide for more robust modeling in thermal ecology since any conclusions derived from it will be based on observed data rather than using theoretical assumptions. It may also provide constraints for systems biology model predictions and provide insights in physiology. The spectrum has a Δ-shape with a sharp peak at around 42°C. At higher temperatures up to 60°C there was a gap of attenuated growth rates. We found another peak at 67°C and a steady decline in maximum rates thereafter. By using Bayesian quantile regression to summarise and explore the data we were able to conclude that the gap represented an actual biological transition between mesophiles and thermophiles that we term the Mesophile-Thermophile Gap (MTG. We have not identified any organism that grows above the maximum rate of the spectrum. We used a thermodynamic model to recover the Δ-shape, suggesting that the growth rate limits arise from a trade-off between activity and stability of proteins. The spectrum provides underpinning principles that will find utility in models concerned with the thermal responses of biological processes.

  3. An expanding locally anisotropic (ELA) metric describing matter in an expanding universe

    Energy Technology Data Exchange (ETDEWEB)

    Castelo Ferreira, P., E-mail: pedro.castelo.ferreira@ist.utl.p [CENTRA-Instituto Superior Tecnico, Av. Rovisco Pais 1, 1100-001 Lisboa (Portugal); U. Lusofona de Humanidades e Tecnologia-Eng. Electrotecnica, Campo Grande 376, 1749-024 Lisboa (Portugal)

    2010-02-08

    It is suggested an expanding locally anisotropic metric (ELA) ansatz describing matter in a flat expanding universe which interpolates between the Schwarzschild (SC) metric near point-like central bodies of mass M and the Robertson-Walker (RW) metric for large radial coordinate: ds{sup 2}=Zc{sup 2}dt{sup 2}-1/Z (dr{sub 1}-(Hr{sub 1})/c Z{sup (alpha)/2+1/2} cdt){sup 2}-r{sub 1}{sup 2}dOMEGA, where Z=1-U{sub SC} with U{sub SC}=2GM/(c{sup 2}r{sub 1}), G is the Newton constant, c is the speed of light, H=H(t)=a{sup .}/a is the time-dependent Hubble rate, dOMEGA=dtheta{sup 2}+sin{sup 2}thetadphi{sup 2} is the solid angle element, a is the universe scale factor and we are employing the coordinates r{sub 1}=ar, being r the radial coordinate for which the RW metric is diagonal. For constant exponent alpha=alpha{sub 0}=0 it is retrieved the isotropic McVittie (McV) metric and for alpha=alpha{sub 0}=1 it is retrieved the locally anisotropic Cosmological-Schwarzschild (SCS) metric, both already discussed in the literature. However it is shown that only for constant exponent alpha=alpha{sub 0}>1 exists an event horizon at the SC radius r{sub 1}=2GM/c{sup 2} and only for alpha=alpha{sub 0}>=3 space-time is singularity free for this value of the radius. These bounds exclude the previous existing metrics, for which the SC radius is a naked extended singularity. In addition it is shown that for alpha=alpha{sub 0}>5 space-time is approximately Ricci flat in a neighborhood of the event horizon such that the SC metric is a good approximation in this neighborhood. It is further shown that to strictly maintain the SC mass-pole at the origin r{sub 1}=0 without the presence of more severe singularities it is required a radial coordinate-dependent correction to the exponent alpha(r{sub 1})=alpha{sub 0}+alpha{sub 1}2GM/(c{sup 2}r{sub 1}) with a negative coefficient alpha{sub 1}<0. The energy-momentum density, pressures and equation of state are discussed.

  4. Self-Expanding, Tough Biodegradable Elastomers for Wound Stasis

    Science.gov (United States)

    2015-08-06

    the civilian setting, with no effective therapies available at point of injury. We previously reported that a self- expanding polyurethane foam...setting, with no effective therapies available at point of injury. We previously reported that a self-expanding polyurethane foam significantly...care in accor- dance with the Guide of the Care and Use of Laboratory Ani- mals (Health, 2011 #17). 2.2. Instrumentation and monitoring All swine (n

  5. Twin screw two-phase expanders in large chiller units

    Energy Technology Data Exchange (ETDEWEB)

    Smith, I.K.; Stosic, N.; Aldis, C.A.; Kovacevic, A. [City Univ., Dept. of Mechanical Engineering and Aeronautics, London (United Kingdom)

    1999-07-01

    An investigation was carried out to determine the feasibility of the use of a twin screw expander as a throttle valve replacement in a 500 ton chiller. The aim was to produce a demonstration unit with an overall machine adiabatic efficiency of not less that 70%. The efficiency target was effectively met but further analytical work is needed to predict mass flow rates reliably and hence the exact machine size required when refrigerant enters the expander as subcooled liquid. (Author)

  6. Unsteady Viscous Flow over an Expanding Stretching Cylinder

    Institute of Scientific and Technical Information of China (English)

    方铁钢; 章骥; 钟永芳; 陶华

    2011-01-01

    We study the viscous How over an expanding stretching cylinder. The solution is exact to the Navier-Stokes equations. The stretching velocity of the cylinder is proportional to the axial distance from the origin and decreases with time. There exists a unique solution for the How with all the studied values of Reynolds number and the unsteadiness parameter. Reversal Hows exist for an expanding stretching cylinder. The velocity decays faster for a larger Reynolds number and a more rapidly expanding cylinder.%We study the viscous flow over an expanding stretching cylinder.The solution is exact to the Navier-Stokes equations.The stretching velocity of the cylinder is proportional to the axial distance from the origin and decreases with time.There exists a unique solution for the flow with all the studied values of Reynolds number and the unsteadiness parameter.Reversal flows exist for an expanding stretching cylinder.The velocity decays faster for a larger Reynolds number and a more rapidly expanding cylinder.

  7. Can the tinnitus spectrum identify tinnitus subgroups?

    NARCIS (Netherlands)

    Heijneman, Karin M.; de Kleine, Emile; Wiersinga-Post, Esther; van Dijk, Pim

    2013-01-01

    The tinnitus spectrum is a psycho-acoustic metric of tinnitus. Previous work found a tight relation between the spectrum and the tone audiogram. This suggests that the spectrum and the audiogram provide essentially the same information, and the added value of the spectrum is limited. In order to tes

  8. Density Spectrums from Kinetic Inflations

    CERN Document Server

    Hwang, J

    1996-01-01

    The pole-like accelerated expansion stages purely driven by the coupling between the gravity and the dilaton field without referring to the potential term can be realized in a class of generalized gravity theories. We consider three such scenarios based on the scalar-tensor gravity, the induced gravity and the string theory. Quantum fluctuations during the expansion stages (including more general situations) can be derived in exact analytic forms. Assuming that the pole-like acceleration stage provides a viable inflation scenario in the early universe we derive the generated classical density spectrums. The generated classical density field shows a generic tilted spectrum with $n \\simeq 4$ which differs from the observed spectrum supporting $n \\simeq 1$.

  9. The Sun's Photospheric Convection Spectrum

    CERN Document Server

    Hathaway, David H; Norton, Aimee A; Kitiashvili, Irina

    2015-01-01

    Spectra of the cellular photospheric flows are determined from full-disk Doppler velocity observations acquired by the Helioseismic and Magnetic Imager (HMI) instrument on the Solar Dynamics Observatory (SDO) spacecraft. Three different analysis methods are used to separately determine spectral coefficients representing the poloidal flows, the toroidal flows, and the radial flows. The amplitudes of these spectral coefficients are constrained by simulated data analyzed with the same procedures as the HMI data. We find that the total velocity spectrum rises smoothly to a peak at a wavenumber of about 120 (wavelength of about 35 Mm), which is typical of supergranules. The spectrum levels off out to wavenumbers of about 400, and then rises again to a peak at a wavenumber of about 3500 (wavelength of about 1200 km), which is typical of granules. The velocity spectrum is dominated by the poloidal flow component (horizontal flows with divergence but no curl) at wavenumbers above 30. The toroidal flow component (hori...

  10. Widespread atypical vascular lesions of the skin after whole-body electron beam therapy: expanding the clinical spectrum.

    Science.gov (United States)

    Sinclair, Werner

    2013-02-01

    Atypical vascular lesion of the skin is an uncommon usually benign condition, thus far reported almost exclusively from mammary skin after radiotherapy for carcinoma of the breast. Some clinical and histological overlap exists with early angiosarcoma, which can also occur on irradiated skin. The lesions are divided into vascular and lymphatic types, the first representing a higher risk for development of angiosarcoma and the latter being more common. This article reports a rare case of widespread, progressive, vascular-type atypical vascular lesion after repeated whole-body electron beam irradiation administered as treatment for mycosis fungoides.

  11. IgG4 related sclerosing mastitis: expanding the morphological spectrum of IgG4 related diseases.

    Science.gov (United States)

    Chougule, Abhijit; Bal, Amanjit; Das, Ashim; Singh, Gurpreet

    2015-01-01

    IgG4 related disease (IgG4RD) is a recently recognised condition characterised by mass forming lesions associated with storiform fibrosis, obliterative phlebitis, lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells and elevated serum IgG4 levels. Although rare, mammary involvement has been reported as IgG4 related sclerosing mastitis, the morphological counterpart of a growing family of IgG4 related diseases. A total of 17 cases belonging to mass forming benign inflammatory breast lesions such as plasma cell mastitis, granulomatous lobular mastitis, non-specific mastitis and inflammatory pseudotumour were investigated as a possible member of IgG4 related sclerosing mastitis. Clinical, radiological, histopathological and immunohistochemistry findings were noted in all cases. Cases diagnosed as inflammatory pseudotumour showed all the histopathological features of IgG4RD along with increased number of IgG4 positive plasma cells and IgG4/IgG ratio >40%. However, only a few IgG4 positive cells were seen in plasma cell mastitis, granulomatous lobular mastitis and non-specific mastitis cases. These cases also did not fulfill the morphological criteria for the diagnosis of IgG4 related diseases. IgG4RD should be excluded in plasma cell rich lesions diagnosed on core biopsies by IgG4 immunostaining. This can avoid unnecessary surgery as IgG4 related diseases respond to simple and effective steroid treatment.

  12. Use of Audio Cuing to Expand Employment Opportunities for Adolescents with Autism Spectrum Disorders and Intellectual Disabilities

    Science.gov (United States)

    Allen, Keith D.; Burke, Raymond V.; Howard, Monica R.; Wallace, Dustin P.; Bowen, Scott L.

    2012-01-01

    We evaluated audio cuing to facilitate community employment of individuals with autism and intellectual disability. The job required promoting products in retail stores by wearing an air-inflated WalkAround[R] costume of a popular commercial character. Three adolescents, ages 16-18, were initially trained with video modeling. Audio cuing was then…

  13. The expanding spectrum of pediatric anti-glutamic acid decarboxylase antibody mediated CNS disease - a chance association?

    Institute of Scientific and Technical Information of China (English)

    Deepak Menon; Ramshekhar N Menon; Hardeep Kumar; Ashalatha Radhakrishnan; Sudheeran Kannoth; Muralidharan Nair; Sanjeev Thomas

    2016-01-01

    Central nervous system autoimmunity in the pediatric age group represents an evolving constellation of various syndromes distinct from the adult age group. One of the rarely described pathogenic auto-antibodies (ab) is the one directed against glutamic acid decarboxylase (GAD). While its pathogenic role is controversial, literature concerning adult patients abounds with heterogeneous presentations with epilepsy often as part of limbic encephalitis or chronic temporal lobe epilepsy and cerebellar ataxia accompanying endocrinopathies or paraneoplastic disorders. Diagnosis is often delayed until late adulthood. The authors report hitherto under-reported syndromes in the pediatric age group. The ifrst case was a 3-year-old boy with sub-acute myoclonus-ataxia following a lfu-like illness akin to para-infectious cerebellitis. The second case was a 7-year-old girl with long-standing chronic extratemporal partial epilepsy and electrical status epilepticus in sleep (ESES) with right hemiparesis and developmental delay. Investigations revealed two-four fold elevations in titres of GAD-65-ab. The absence of systemic diseases like diabetes and the dramatic clinical response to steroids as well as intravenous immunoglobulin in both the cases argued for GAD-ab mediated neuronal injury rather than a chance association. The concern exists regarding other potentially co-existent auto-ab to gamma-amino butyric acid and glycine receptors, and demonstration of intrathecal synthesis of GAD-ab would be ideal. This entity should be contemplated in children presenting with acute/sub-acute onset episodic or progressive ataxia or refractory cryptogenic focal epilepsy syndromes, epileptic encephalopathy such as ESES and worsening neurological deifcits. These children ought to be maintained on regular follow-up for monitoring evolution of other autoimmune disorders in adult life.

  14. Expanding the Therapeutic Spectrum of Artemisinin:Activity Against Infectious Diseases Beyond Malaria and Novel Pharmaceutical Developments

    Institute of Scientific and Technical Information of China (English)

    Thomas Efferth; Jose J.G. Marin; Marta R. Romero; Anna Rita Bilia; Ahmed Galal Osman; Mahmoud ElSohly; Michael Wink; Rudolf Bauer; Ikhlas Khan; Maria Camilla Bergonzi

    2016-01-01

    The interest of Western medicine in Traditional Chinese Medicine (TCM) as a source of drug leads/new drugs to treat diseases without available efficient therapies has been dramatical y augmented in the last decades by the extensive work and the outstanding findings achieved within this kind of medicine. The practice of TCM over thousands of years has equipped scientists with substantial experience with hundreds of plants that led to the discovery of artemisinin (qinghaosu), which is extracted from the medicinal plant Artemisia annua L. (qinghao). The unexpected success of artemisinin in combating malaria has drawn strong attention from the scientific community towards TCM. Artemisinin was discovered by Youyou Tu in 1972. Since then, several novel pharmacological activities based on the well-known properties of the sesquiterpene lactone structure with the oxepane ring and an endoperoxide bridge have been unravelled. Beyond malaria, artemisinin and its derivatives (artemisinins) exert profound activities towards other protozoans (Leishmania, Trypanosoma, amoebas, Neospora caninum, and Eimeria tenella), trematodes (Schistosoma, liver flukes), and viruses (human cytomegalovirus, hepatitis B and C viruses). Less clear is the effect against bacteria and fungi. Based on the promising results of artemisinin and the first generation derivatives (artesunate, artemether, arteether), novel drug development strategies have been pursued. These included the synthesis of acetal-and non-acetal-type artemisinin dimeric molecules as wel as developing nanotechnological approaches, e.g. artemisinin-based liposomes, niosomes, micel es, solid lipid nanocarriers, nanostructured lipid carriers, nanoparticles, fullerenes and nanotubes. The current review presents an overview on different aspects of artemisinins, including sources, chemistry, biological/pharmacological properties, types of infectious pathogens that are susceptible to artemisinins in vitro and in vivo, in addition to the advancement in their drug delivery systems utilizing pharmaceutical technology. It would be expected that different therapeutic strategies based on the second and third generation artemisinin derivatives and artemisinin-based drug technologies would be available in the near future to treat specific infectious diseases.

  15. Compressive Wideband Spectrum Sensing for Fixed Frequency Spectrum Allocation

    CERN Document Server

    Liu, Yipeng

    2010-01-01

    Too high sampling rate is the bottleneck to wideband spectrum sensing for cognitive radio (CR). As the survey shows that the sensed signal has a sparse representation in frequency domain in the mass, compressed sensing (CS) can be used to transfer the sampling burden to the digital signal processor. An analog to information converter (AIC) can randomly sample the received signal with sub-Nyquist rate to obtained the random measurements. Considering that the static frequency spectrum allocation of primary radios means the bounds between different primary radios is known in advance, here we incorporate information of the spectrum boundaries between different primary user as a priori information to obtain a mixed l2/l1 norm denoising operator (MNDO). In the MNDO, the estimated power spectrum density (PSD) vector is divided into block sections with bounds corresponding different allocated primary radios. Different from previous standard l1-norm constraint on the whole PSD vector, a sum of the l2 norm of each sect...

  16. Luminosity Spectrum Reconstruction at Linear Colliders

    CERN Document Server

    Poss, Stéphane

    2014-01-01

    A good knowledge of the luminosity spectrum is mandatory for many measurements at future e+e- colliders. As the beam-parameters determining the luminosity spectrum cannot be measured precisely, the luminosity spectrum has to be measured through a gauge process with the detector. The measured distributions, used to reconstruct the spectrum, depend on Initial State Radiation, cross-section, and Final State Radiation. To extract the basic luminosity spectrum, a parametric model of the luminosity spectrum is created, in this case the spectrum at the 3 TeV Compact Linear Collider (CLIC). The model is used within a reweighting technique to extract the luminosity spectrum from measured Bhabha event observables, taking all relevant effects into account. The centre-of-mass energy spectrum is reconstructed within 5% over the full validity range of the model. The reconstructed spectrum does not result in a significant bias or systematic uncertainty in the exemplary physics benchmark process of smuon pair production.

  17. Dynamic spectrum auction in wireless communication

    CERN Document Server

    Chen, Yanjiao

    2015-01-01

    This brief explores current research on dynamic spectrum auctions, focusing on fundamental auction theory, characteristics of the spectrum market, spectrum auction architecture and possible auction mechanisms. The brief explains how dynamic spectrum auctions, which enable new users to gain spectrum access and existing spectrum owners to obtain financial benefits, can greatly improve spectrum efficiency by resolving the artificial spectrum shortage. It examines why operators and users face significant challenges due to specialty of the spectrum market and the related requirements imposed on the auction mechanism design. Concise and up-to-date, Dynamic Spectrum Auction in Wireless Communication is designed for researchers and professionals in computer science or electrical engineering. Students studying networking will also find this brief a valuable resource.

  18. Spread Spectrum Mobile Radio Communications.

    Science.gov (United States)

    1984-09-15

    seconds, yielding Z Y a =XMZ m M .-m The sequence of operacions is illustrated by the matrices of figures ’--3- and 1-3b. Each matrix is either a...sequence of K-bit numbers (code word, address, detection matrix) or a frequency-time spectrogram (traasmit spectrum, receive spectrum). The matrices pertain...and a reduced rank-sum receiver. In figure 3-1, we show the operation of a MRSR by means of matrices . 3-1 Reduced Rank-Sum Receiver With the values of

  19. Autism Spectrum Disorders in Iran

    OpenAIRE

    Mohammadi, Mohammad Reza; Salmanian, Maryam; Akhondzadeh, Shahin

    2011-01-01

    How to Cite this Article: Mohammadi MR, Salmanian M, Akhondzadeh Sh. Autism Spectrum Disorders in Iran. Iranian Journal of Child Neurology2011;5(4):1-9.ObjectiveAutistic disorder, Asperger syndrome, and PDD-Not Otherwise Specified are subsets of autism spectrum disorders (ASDs), which are characterized by impairments in social communication and stereotyped behavior. This article reviews the prevalence, etiology, diagnosis, and treatment of ASDs in Iran.Materials & MethodsWe searched PubMe...

  20. A recurrent germline BAP1 mutation and extension of the BAP1 tumor predisposition spectrum to include basal cell carcinoma

    DEFF Research Database (Denmark)

    Wadt, K A W; Aoude, L G; Johansson, P;

    2015-01-01

    We report four previously undescribed families with germline BAP1 mutations and expand the clinical phenotype of this tumor syndrome. The tumor spectrum in these families is predominantly uveal malignant melanoma (UMM), cutaneous malignant melanoma (CMM) and mesothelioma, as previously reported f...

  1. On the Mechanism for Breaks in the Cosmic Ray Spectrum

    Science.gov (United States)

    Malkov, Mikhail

    2011-11-01

    Recent observations of galactic supernova remnants by the Fermi spacecraft observatory strongly support the idea that the bulk of galactic cosmic rays are accelerated in such remnants by a Fermi mechanism, also known as diffusive shock acceleration. However, the remnants most visible in gamma rays expand into weakly ionized dense gas, and so a significant revision of the basic mechanism is required. In this talk, I provide the necessary modifications and demonstrate that strong ion- neutral collisions in the remnant lead to steepening of the energy spectrum of accelerated particles by exactly one power. The spectral break is caused by Alfven wave evanescence leading to fractional particle losses. The gamma-ray spectrum generated in collisions of the accelerated protons with the ambient gas is also calculated and successfully fitted to the Fermi data. The parent proton spectrum is best represented by a classical test particle power law E-2, steepening to E-3 at Ebr=7GeV due to deteriorated particle confinement.

  2. Evidence for Expanding Superbubbles in a Galaxy at z=0.7443

    CERN Document Server

    Bond, N A; Charlton, J C; Vogt, S S; Bond, Nicholas A.; Churchill, Chris W.; Charlton, Jane C.

    2001-01-01

    The intervening z=0.7443 Mg II absorption system in the spectrum of MC 1331+170 shows an unusual series of line pairs, each with velocity separations of 30 km/s. These lines could be explained as the shells of expanding superbubbles residing in the outer regions of an edge-on spiral galaxy visible in the optical image of the MC 1331+170 field. The color and brightness of this galaxy make it the most likely candidate z=0.7443 absorber, though two other galaxies in the quasar field could also be contributing to the Mg II absorption profile. Kinematic models of absorption from compact groups and galaxy pairs produce profiles largely inconsistent with the observed Mg II spectrum. Superbubbles would naturally generate more regular structures such as those observed. Photoionization models of the superbubble shell are consistent with the observed profile for many realistic physical conditions. In a pure superbubble model, the large velocity spread of the Mg II absorption system is inconsistent with the expected spre...

  3. The Development of Kolmogoroff-Like Power Spectra in the Expanding Solar Wind

    Science.gov (United States)

    Goldstein, M. L.; Roberts, D. A.; Ghosh, S.; Deane, A. E.

    1998-01-01

    Power spectra of the solar wind fluctuations consistently exhibit a -5/3 power-law slope consistent with the idea that the medium is undergoing a turbulent cascade as seen in ordinary fluids. This is surprising both because the radial streams and the magnetic field threading the plasma will induce anisotropies and because the expansion of the wind will tend to lead to the suppression of nonlinear cascades. These conditions violate the assumptions used by Kolmogoroff to derive the -5/3 law. We have studied this issue using a compressible IMHD code in spherical coordinates and have shown that a -5/3 spectrum results from a broad-band flat-spectrum input condition that is sheared and distorted by a current sheet as the wind expands. We determine spectra from time series taken at selected points in the domain as is done with observational spacecraft data. The spectra are very like those we have seen in nonexpanding runs and exhibit evolution and compressive characteristics very similar to those seen in observations. We will report on these results in addition to a new set of runs intended to constrain the necessary and sufficient conditions for the spectra to have this form. The simulation also allows us to examine the anisotropy for the spectra to attempt to determine why the result of an isotropic magnetofluid is obtained in a highly anisotropic situation.

  4. [Autism spectrum disorders in adults

    NARCIS (Netherlands)

    Kan, C.C.; Buitelaar, J.K.; Gaag, R.J. van der

    2008-01-01

    Early infantile autism' as defined by Kanner has grown into a spectrum of autistic disorders. The recognition of Asperger's disorder and of pervasive developmental disorder not otherwise specified (PDD-NOS), has led to increased demand for appropriate diagnostic assessment of autism in adults. The e

  5. Primordial power spectrum from Planck

    CERN Document Server

    Hazra, Dhiraj Kumar; Souradeep, Tarun

    2014-01-01

    Using modified Richardson-Lucy algorithm we reconstruct the primordial power spectrum (PPS) from Planck Cosmic Microwave Background (CMB) temperature anisotropy data. In our analysis we use different combinations of angular power spectra from Planck to reconstruct the shape of the primordial power spectrum and locate possible features. Performing an extensive error analysis we found the dip near $\\ell\\sim750-850$ represents the most prominent feature in the data. Feature near $\\ell\\sim1800-2000$ is detectable with high confidence only in 217 GHz spectrum and is apparently consequence of a small systematic as described in the revised Planck 2013 papers. Fixing the background cosmological parameters and the foreground nuisance parameters to their best fit baseline values, we report that the best fit power law primordial power spectrum is consistent with the reconstructed form of the PPS at 2$\\sigma$ C.L. of the estimated errors (apart from the local features mentioned above). As a consistency test, we found the...

  6. Diophantine approximation and automorphic spectrum

    CERN Document Server

    Ghosh, Anish; Nevo, Amos

    2010-01-01

    The present paper establishes qunatitative estimates on the rate of diophantine approximation in homogeneous varieties of semisimple algebraic groups. The estimates established generalize and improve previous ones, and are sharp in a number of cases. We show that the rate of diophantine approximation is controlled by the spectrum of the automorphic representation, and is thus subject to the generalised Ramanujan conjectures.

  7. The low lying glueball spectrum

    Energy Technology Data Exchange (ETDEWEB)

    Adam Szczepaniak; Eric Swanson

    2003-12-18

    The complete low-lying positive charge conjugation glueball spectrum is obtained from QCD. The formalism relies on the construction of an efficient quasiparticle gluon basis for Hamiltonian QCD in Coulomb gauge. The resulting rapidly convergent Fock space expansion is exploited to derive quenched low-lying glueball masses with no free parameters which are in remarkable agreement with lattice gauge theory.

  8. Autism Spectrum Disorders and Epigenetics

    Science.gov (United States)

    Grafodatskaya, Daria; Chung, Brian; Szatmari, Peter; Weksberg, Rosanna

    2010-01-01

    Objective: Current research suggests that the causes of autism spectrum disorders (ASD) are multifactorial and include both genetic and environmental factors. Several lines of evidence suggest that epigenetics also plays an important role in ASD etiology and that it might, in fact, integrate genetic and environmental influences to dysregulate…

  9. Catatonia and Autistic Spectrum Disorders

    Science.gov (United States)

    Hare, Dougal Julian; Malone, Caroline

    2004-01-01

    The phenomenon of catatonic-like states in people with autistic spectrum disorders is discussed in the context of current knowledge about catatonia as it occurs in severe mental illness and, less frequently documented, in conjunction with developmental disorders. The existing literature on catatonic-like states in people with autistic spectrum…

  10. Autism Spectrum Disorders and Epigenetics

    Science.gov (United States)

    Grafodatskaya, Daria; Chung, Brian; Szatmari, Peter; Weksberg, Rosanna

    2010-01-01

    Objective: Current research suggests that the causes of autism spectrum disorders (ASD) are multifactorial and include both genetic and environmental factors. Several lines of evidence suggest that epigenetics also plays an important role in ASD etiology and that it might, in fact, integrate genetic and environmental influences to dysregulate…

  11. Neurofeedback in Autism Spectrum Disorders

    Science.gov (United States)

    Holtmann, Martin; Steiner, Sabina; Hohmann, Sarah; Poustka, Luise; Banaschewski, Tobias; Bolte, Sven

    2011-01-01

    Aim: To review current studies on the effectiveness of neurofeedback as a method of treatment of the core symptoms of autism spectrum disorders (ASD). Method: Studies were selected based on searches in PubMed, Ovid MEDLINE, EMBASE, ERIC, and CINAHL using combinations of the following keywords: "Neurofeedback" OR "EEG Biofeedback" OR "Neurotherapy"…

  12. Army PM UAS Spectrum Update

    Science.gov (United States)

    2012-07-01

    35.15GHz Sierra Nevada Corporation ( SNC ) 1 Aircraft (Wing) J/F-12 06982 TALS TS - Ground Unit RX 34.85 to 35.15GHz TX 34.78 to 35.08GHz Sierra...Nevada Corporation ( SNC ) 2 Runway J/F-12 06982 UNCLASSIFIED UNCLASSIFIED Gray Eagle RF Spectrum Requirements 23 System Component

  13. [Autism spectrum disorders in adults

    NARCIS (Netherlands)

    Kan, C.C.; Buitelaar, J.K.; Gaag, R.J. van der

    2008-01-01

    Early infantile autism' as defined by Kanner has grown into a spectrum of autistic disorders. The recognition of Asperger's disorder and of pervasive developmental disorder not otherwise specified (PDD-NOS), has led to increased demand for appropriate diagnostic assessment of autism in adults. The

  14. Generalized eigenvalue based spectrum sensing

    KAUST Repository

    Shakir, Muhammad

    2012-01-01

    Spectrum sensing is one of the fundamental components in cognitive radio networks. In this chapter, a generalized spectrum sensing framework which is referred to as Generalized Mean Detector (GMD) has been introduced. In this context, we generalize the detectors based on the eigenvalues of the received signal covariance matrix and transform the eigenvalue based spectrum sensing detectors namely: (i) the Eigenvalue Ratio Detector (ERD) and two newly proposed detectors which are referred to as (ii) the GEometric Mean Detector (GEMD) and (iii) the ARithmetic Mean Detector (ARMD) into an unified framework of generalize spectrum sensing. The foundation of the proposed framework is based on the calculation of exact analytical moments of the random variables of the decision threshold of the respective detectors. The decision threshold has been calculated in a closed form which is based on the approximation of Cumulative Distribution Functions (CDFs) of the respective test statistics. In this context, we exchange the analytical moments of the two random variables of the respective test statistics with the moments of the Gaussian (or Gamma) distribution function. The performance of the eigenvalue based detectors is compared with the several traditional detectors including the energy detector (ED) to validate the importance of the eigenvalue based detectors and the performance of the GEMD and the ARMD particularly in realistic wireless cognitive radio network. Analytical and simulation results show that the newly proposed detectors yields considerable performance advantage in realistic spectrum sensing scenarios. Moreover, the presented results based on proposed approximation approaches are in perfect agreement with the empirical results. © 2012 Springer Science+Business Media Dordrecht.

  15. Self-expanding/shrinking structures by 4D printing

    Science.gov (United States)

    Bodaghi, M.; Damanpack, A. R.; Liao, W. H.

    2016-10-01

    The aim of this paper is to create adaptive structures capable of self-expanding and self-shrinking by means of four-dimensional printing technology. An actuator unit is designed and fabricated directly by printing fibers of shape memory polymers (SMPs) in flexible beams with different arrangements. Experiments are conducted to determine thermo-mechanical material properties of the fabricated part revealing that the printing process introduced a strong anisotropy into the printed parts. The feasibility of the actuator unit with self-expanding and self-shrinking features is demonstrated experimentally. A phenomenological constitutive model together with analytical closed-form solutions are developed to replicate thermo-mechanical behaviors of SMPs. Governing equations of equilibrium are developed for printed structures based on the non-linear Green-Lagrange strain tensor and solved implementing a finite element method along with an iterative incremental Newton-Raphson scheme. The material-structural model is then applied to digitally design and print SMP adaptive lattices in planar and tubular shapes comprising a periodic arrangement of SMP actuator units that expand and then recover their original shape automatically. Numerical and experimental results reveal that the proposed planar lattice as meta-materials can be employed for plane actuators with self-expanding/shrinking features or as structural switches providing two different dynamic characteristics. It is also shown that the proposed tubular lattice with a self-expanding/shrinking mechanism can serve as tubular stents and grippers for bio-medical or piping applications.

  16. Strength analysis of expandable tubulars for well applications

    Energy Technology Data Exchange (ETDEWEB)

    Aguiar, A.C.C. [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil); Fonseca, C.E. [PETROBRAS S.A., Rio de Janeiro, RJ (Brazil); Netto, T.A. [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Coordenacao dos Programas de Pos-graduacao de Engenharia (COPPE)

    2008-07-01

    Solid expandable tube technology has many advantages when compared to conventional wells. The expansion of tubes in situ allows developing reserves in many of the challenging scenarios found in oil industry, as pre-salt layer, HPHT wells, deep reservoirs, or ultra-deep water. Besides, this procedure has good compatibility with directional and horizontal wells and facilitates side-tracks operations. Although the expansion of tubes is very attractive, a better understanding of its influence on the tube mechanical strength is necessary. In this work, experimental tests and numerical analyses were performed in order to determine the effect of parameters such as diameter-to-thickness ratio and expansion rate on the collapse resistance of expandable tubes. An experimental apparatus was designed and built to reproduce full-scale tube expansion. Three 2 meter long specimens were expanded 10% their original diameters and subjected to hydrostatic pressure inside a vessel until collapse. Three non-expanded tubes were also tested for comparison. At the same time, non-linear numerical models were developed using the finite element method. After calibration, they were used to further analyze the mechanical behavior of solid expandable tubes and the influence of expansion on its resistance against collapse. (author)

  17. Expanded tenses in the old English orosius a syntactic strengthening*

    Directory of Open Access Journals (Sweden)

    Frančiška Trobevšek Drobnak

    1990-12-01

    Full Text Available The present chapter reports the investigation into certain aspects of the periphrastic construction to be +present participle (e.g. NE: "he is teaching"; OE. "he is laerende" viewed as an example of a syntactic strengthening. The construc­ tion is usually referred to as "continuous tenses/form" or "progressive tenses/form", whereas Nickel (1966 uses "expanded form". Coming closest to this latter term, the "expanded tenses" employed here seems a convenient label for two reasons: a  the use of expanded tenses is not restricted to the expression of verbal aspect (Aspekt or mode of verbal action (Aktionsart, which is implied by the use of either the term "continuous  tenses/form" or the term "progressive tenses/form"; b  the expanded tenses are integrated into the English tense system, in the sense that they can be substituted  with the respective non-expanded tenses without any change in the syntax of the clause, e.g.

  18. Theoretically Founded Optimization of Auctioneer's Revenues in Expanding Auctions

    Science.gov (United States)

    Rabin, Jonathan; Shehory, Onn

    The expanding auction is a multi-unit auction which provides the auctioneer with control over the outcome of the auction by means of dynamically adding items for sale. Previous research on the expanding auction has provided a numeric method to calculate a strategy that optimizes the auctioneer's revenue. In this paper, we analyze various theoretical properties of the expanding auction, and compare it to VCG, a multi-unit auction protocol known in the art. We examine the effects of errors in the auctioneer's estimation of the buyers' maximal bidding values and prove a theoretical bound on the ratio between the revenue yielded by the Informed Decision Strategy (IDS) and the post-optimal strategy. We also analyze the relationship between the auction step and the optimal revenue and introduce a method of computing this optimizing step. We further compare the revenues yielded by the use of IDS with an expanding auction to those of the VCG mechanism and determine the conditions under which the former outperforms the latter. Our work provides new insight into the properties of the expanding auction. It further provides theoretically founded means for optimizing the revenue of auctioneer.

  19. Theoretical investigation of flash vaporisation in a screw expander

    Science.gov (United States)

    Vasuthevan, Hanushan; Brümmer, Andreas

    2017-08-01

    In the present study flash vaporisation of liquid injection in a twin screw expander for a Trilateral Flash Cycle (TFC) is examined theoretically. The TFC process comprises a pressure increase in the working fluid, followed by heating the liquid close to boiling point. The hot liquid is injected into the working chamber of a screw expander. During this process the pressure of the liquid drops below the saturation pressure, while the temperature of the liquid remains virtually constant. Hence the liquid is superheated and in a metastable state. The liquid jet seeks to achieve a stable state in thermodynamic equilibrium and is therefore partially vaporised. This effect is referred to as flash vaporisation. Accordingly, a two-phase mixture, consisting of vapour and liquid, exists in the working chamber. Thermodynamic simulations were carried out using water as the working fluid for representative screw expander geometry. The simulations presented are performed from two different aspects during the filling process of a screw expander. The first case is the vaporisation of the injected liquid in a state of thermodynamic equilibrium, whereby the two-phase mixture is treated entirely as a compressible and homogeneous gas. The second case considers flashing efficiency. It describes the quantity of flashed vapour and consists of a liquid and vapour domain. Both models are compared and analysed with respect to the operational behaviour of a screw expander.

  20. Numerical simulation of a twin screw expander for performance prediction

    Science.gov (United States)

    Papes, Iva; Degroote, Joris; Vierendeels, Jan

    2015-08-01

    With the increasing use of twin screw expanders in waste heat recovery applications, the performance prediction of these machines plays an important role. This paper presents a mathematical model for calculating the performance of a twin screw expander. From the mass and energy conservation laws, differential equations are derived which are then solved together with the appropriate Equation of State in the instantaneous control volumes. Different flow processes that occur inside the screw expander such as filling (accompanied by a substantial pressure loss) and leakage flows through the clearances are accounted for in the model. The mathematical model employs all geometrical parameters such as chamber volume, suction and leakage areas. With R245fa as working fluid, the Aungier Redlich-Kwong Equation of State has been used in order to include real gas effects. To calculate the mass flow rates through the leakage paths formed inside the screw expander, flow coefficients are considered as constant and they are derived from 3D Computational Fluid Dynamic calculations at given working conditions and applied to all other working conditions. The outcome of the mathematical model is the P-V indicator diagram which is compared to CFD results of the same twin screw expander. Since CFD calculations require significant computational time, developed mathematical model can be used for the faster performance prediction.

  1. Non-ergodicity for $C^{1}$ Expanding Maps

    CERN Document Server

    Quas, A N

    1993-01-01

    In this paper, we consider the question of existence and uniqueness of absolutely continuous invariant measures for expanding $C^1$ maps of the circle. This is a question which arises naturally from results which are known in the case of expanding $C^k$ maps of the circle where $k\\geq 2$, or even $C^{1+\\epsilon}$ expanding maps of the circle. In these cases, it is known that there exists a unique absolutely continuous invariant probability measure by the so-called `Folklore Theorem'. It follows that this measure is ergodic. It has been shown however that for $C^1$ maps there need not be any such measure. However, this leaves the question of whether there can be more than one such measure for $C^1$ expanding maps of the circle. This is the subject of this paper, and in it, we show that there exists a $C^1$ expanding map of the circle which has more than one absolutely continuous invariant probability measure.

  2. The technique of sand control with expandable screens

    Energy Technology Data Exchange (ETDEWEB)

    Zhao, P. [Petrochina, Liaohe (China). Liaohe Oilfield Co.

    2009-07-01

    Sand production in heavy oil reservoirs can limit the normal production of oil wells. In this study, expandable screens were used as a sand control mechanism by filtering the sand as it entered the wellbore. The screen systems consists of an expandable outer housing, an expandable base pipe and a filtering layer. The screen expands radially through an expandable cone and presses into the casing well. Axial tension is used to shrink the screens radially through a fishing anchor in order to remove them from the well. The lack of a sand ring between the screen and the casing increases the flow area of the oil and reduces flow resistance caused by fine silt blockages. A series of laboratory experiments were conducted to study the expansion and shrinkage properties of the screens. A field test conducted at a well located in the Liaohe oilfield in China demonstrated that good sand control results can be obtained without the need for pump checking. It was concluded that the sand control method is easy to use and provides good sand control results in large open flow areas. 2 refs., 2 tabs., 3 figs.

  3. Spectrum Trading in India and 5G

    DEFF Research Database (Denmark)

    Tripathi, Purnendu; Prasad, Ramjee

    2013-01-01

    Currently radio spectrum is largely managed through Command and Control method. Public mobile services require spectrum below 3 GHz for providing cost effective services. The existing method has created artificial shortage of spectrum especially below 3 GHz. Spectrum trading is a new concept...... in which service providers are permitted to purchase spectrum from the market to fulfil their requirements. Spectrum trading has not yet been permitted in India. This paper provides an overview of possibilities of spectrum trading in India and concludes that necessary ingredients are present in India...... for spectrum trading and it could provide a boost to the Indian telecom sector. Further, it will also discuss spectrum issue related with 5G in the direction of millimeter waves....

  4. An Expanded CAG Repeat in Huntingtin Causes +1 Frameshifting.

    Science.gov (United States)

    Saffert, Paul; Adamla, Frauke; Schieweck, Rico; Atkins, John F; Ignatova, Zoya

    2016-08-26

    Maintenance of triplet decoding is crucial for the expression of functional protein because deviations either into the -1 or +1 reading frames are often non-functional. We report here that expression of huntingtin (Htt) exon 1 with expanded CAG repeats, implicated in Huntington pathology, undergoes a sporadic +1 frameshift to generate from the CAG repeat a trans-frame AGC repeat-encoded product. This +1 recoding is exclusively detected in pathological Htt variants, i.e. those with expanded repeats with more than 35 consecutive CAG codons. An atypical +1 shift site, UUC C at the 5' end of CAG repeats, which has some resemblance to the influenza A virus shift site, triggers the +1 frameshifting and is enhanced by the increased propensity of the expanded CAG repeats to form a stem-loop structure. The +1 trans-frame-encoded product can directly influence the aggregation of the parental Htt exon 1.

  5. Sea Level Enigmatic Rising - New Perspectives from an Expanding Globe

    CERN Document Server

    Scalera, Giancarlo

    2015-01-01

    In the expanding Earth framework it is possible to find additional phenomena that could contribute in a proper way to the water balance and general tectonic eustatism involved in the sea lever rising. Recent compilations seems to leave unexplained up to 12 cm/century of sea rising, and possible solutions invoking a polar ice shells melting near to the upper limit of the error bars reveal in conflict with the consequent expected decreasing of the Earth angular velocity. It is shown that taking into account possible effects of an expanding Earth, the problem can be initiated towards an appropriate solution, at least as regards the just orders of magnitude. Major effects on sea-level could come from ongoing relaxation of curvature variations that are peculiar for an expanding globe.

  6. Two-dimensional hybrid models of H+-He++ expanding solar wind plasma heating

    Science.gov (United States)

    Ofman, L.; Viñas, A. F.; Maneva, Y.

    2014-06-01

    Preferential heating and acceleration of the solar wind He++ ions compared to protons in fast solar wind streams have been known for decades, thanks to in situ spacecraft measurements at 0.29-5 AU. Turbulent magnetic field fluctuations with approximate power law spectra have been observed as well. However, the exact causes of these processes are still not known due to the lack of detailed information on the magnetic field fluctuations and ion velocity distributions in the acceleration region of the solar wind. Here the collisionless heating processes in expanding solar wind plasma are investigated using 2-D hybrid modeling with parameters appropriate to the heliocentric distance of 10 RS. In this study the ion dynamics is described kinetically, while electrons are treated as a background massless fluid in an expanding solar wind model. The source of free energy for the heating is introduced through an initial nonequilibrium state of the plasma with large He++ ion temperature anisotropy or with super-Alfvénic relative ion drift. We also employ an externally imposed spectrum of magnetic fluctuations in the frequency range below the proton gyroresonant frequency to heat the He++ ions. We investigate the effects of solar wind radial expansion by modeling several values of the expansion rate in a parametric study. We find that the preferential ion heating is attained in both nonexpanding and expanding solar wind models. Thus, the expansion has little effect on the preferential He++ ion heating by the processes considered here. Moreover, the expansion leads to faster evolution of the magnetosonic drift instability, reducing the drift velocity to lower values sooner, and the corresponding generation of the magnetic fluctuations that heat the ions, compared to the nonexpanding case. This is due to the reduction of the perpendicular particle velocities in the expanding (inflated) frame. For cases with little proton perpendicular heating, the solar wind expansion leads to

  7. Modelling the TSZ power spectrum

    Energy Technology Data Exchange (ETDEWEB)

    Bhattacharya, Suman [Los Alamos National Laboratory; Shaw, Laurie D [YALE; Nagai, Daisuke [YALE

    2010-01-01

    The structure formation in university is a hierarchical process. As universe evolves, tiny density fluctuations that existed in the early universe grows under gravitational instability to form massive large scale structures. The galaxy clusters are the massive viralized objects that forms by accreting smaller clumps of mass until they collapse under their self-gravity. As such galaxy clusters are the youngest objects in the universe which makes their abundance as a function of mass and redshift, very sensitive to dark energy. Galaxy clusters can be detected by measuring the richness in optical waveband, by measuring the X-ray flux, and in the microwave sky using Sunyaev-Zel'dovich (SZ) effect. The Sunyaev-Zel'dovich (SZ) effect has long been recognized as a powerful tool for detecting clusters and probing the physics of the intra-cluster medium. Ongoing and future experiments like Atacama Cosmology Telescope, the South Pole Telescope and Planck survey are currently surveying the microwave sky to develop large catalogs of galaxy clusters that are uniformly selected by the SZ flux. However one major systematic uncertainties that cluster abundance is prone to is the connection between the cluster mass and the SZ flux. As shown by several simulation studies, the scatter and bias in the SZ flux-mass relation can be a potential source of systematic error to using clusters as a cosmology probe. In this study they take a semi-analytic approach for modeling the intra-cluster medium in order to predict the tSZ power spectrum. The advantage of this approach is, being analytic, one can vary the parameters describing gas physics and cosmology simultaneously. The model can be calibrated against X-ray observations of massive, low-z clusters, and using the SZ power spectrum which is sourced by high-z lower mass galaxy groups. This approach allows us to include the uncertainty in gas physics, as dictated by the current observational uncertainties, while measuring the

  8. Uniformly expanding vacuum: A possible interpretation of the dark energy

    Science.gov (United States)

    Huang, Peng; Yuan, Fang-Fang

    2016-06-01

    Following the spirit of the equivalence principle, we take a step further to recognize the free fall of the observer as a method to eliminate causes that would lead the perceived vacuum to change its original state. Thus, it is expected that the vacuum should be in a rigid Minkowski state or be uniformly expanding. By carefully investigating the impact on measurement caused by the expansion, we clarify the exact meaning of the uniformly expanding vacuum and find that this proposal may be able to explain the current observations of an accelerating universe.

  9. Supercooling of rapidly expanding quark-gluon plasma

    CERN Document Server

    Zabrodin, E E; Csernai, László P; Stöcker, H; Greiner, W

    1998-01-01

    We reexamine the scenario of homogeneous nucleation of the quark-gluon plasma produced in ultra-relativistic heavy ion collisions. A generalization of the standard nucleation theory to rapidly expanding system is proposed. The nucleation rate is derived via the new scaling parameter $\\lambda_Z$. It is shown that the size distribution of hadronic clusters plays an important role in the dynamics of the phase transition. The longitudinally expanding system is supercooled to about 3-6%, then it is reheated, and the hadronization is completed within 6-10 fm/c, i.e. 5-10 times faster than it was estimated earlier, in a strongly nonequilibrium way.

  10. Chronic expanding hematoma of thorax extended to the neck.

    Science.gov (United States)

    Ueda, Hitoshi; Baba, Hiromitsu; Ondo, Kaoru

    2014-01-01

    We report a rare case of chronic expanding hematoma of thorax extended to the neck. An 83-year-old man with a history of Lucite ball plombage and thoracoplasty of bilateral thorax was admitted with numbness of left upper extremity. In 6 months, left supraclavicular fossa was gradually bulged like tumor. The lesion was diagnosed as chronic expanding hematoma. Surgically, Lucite balls were removed with surrounding hematoma debris and fluid, and neck hematoma, which was slightly communicated to the thorax, was extirpated. We discussed the genesis of this hematoma and its extension to the neck.

  11. Expanding and nonexpanding conformal wormholes, in scalar-tensor theory

    Science.gov (United States)

    Dogru, Melis Ulu; Yilmaz, Ihsan

    2015-06-01

    We study spherically symmetric spacetime with anisotropic fluid in the scalar-tensor theory of gravity based on Lyra geometry. We suggest two different solutions of field equations for the theory by using Casimir effect. Obtained static and nonstatic solutions are similar to nonexpanding Lorentzian wormhole and expanding FRW-type wormhole, respectively. Furthermore, we study singularities of obtained solutions. We emphasize whether the expanding and nonexpanding wormholes conform with Big Rip or Big Crunch scenarios. Also, physical and geometrical properties of the solutions have been discussed.

  12. Sound absorption study of raw and expanded particulate vermiculites

    Science.gov (United States)

    Vašina, Martin; Plachá, Daniela; Mikeska, Marcel; Hružík, Lumír; Martynková, Gražyna Simha

    2016-12-01

    Expanded and raw vermiculite minerals were studied for their ability to absorb sound. Phase and structural characterization of the investigated vermiculites was found similar for both types, while morphology and surface properties vary. Sound waves reflect in wedge-like structure and get minimized, and later are absorbed totally. We found that thanks to porous character of expanded vermiculite the principle of absorption of sound into layered vermiculite morphology is analogous to principle of sound minimization in "anechoic chambers." It was found in this study that the best sound damping properties of the investigated vermiculites were in general obtained at higher powder bed heights and higher excitation frequencies.

  13. Pre-expanded Deep Inferior Epigastric Perforator Flap.

    Science.gov (United States)

    Monsivais, Sharon E; Webster, Nicholas D; Wong, Stacy; Saint-Cyr, Michel H

    2017-01-01

    The deep inferior epigastric perforator (DIEP) flap can be used to cover large defects of the proximal lower extremity, abdominal wall, perineum, vulva, and buttock. Pre-expanding DIEP flaps cause a possible delay phenomenon improving vascularity, decrease donor site morbidity, and increase the area that can be covered. Pre-expansion requires staged procedures, has risk of extrusion and infection, causes temporary contour deformity during the expansion process, and requires a longer course. Pre-expanded DIEP flaps can be a useful flap with proper patient selection and planning. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. A beam expander facility for studying x-ray optics

    DEFF Research Database (Denmark)

    Christensen, Finn Erland; Hornstrup, Allan; Frederiksen, P.

    1992-01-01

    The detailed study of the performance of full scale x-ray optics often requires the illumination of large areas. This paper describes a beam expander facility at the Daresbury Synchrotron Radiation Facility. It combines monochromatization and beam expansion in one dimension. The beam expansion...... is obtained from an extremely asymmetric reflection in a large single crystal of Si. An expansion of a factor of 50 was obtained in one dimension. The expanded beam of ~85 mm is limited only by the crystal size. The facility is installed in a 12-m-long hutch. A specific application, in which a high throughput...

  15. On the Propagation of Light in an Expanding Universe

    Directory of Open Access Journals (Sweden)

    Yuri Heymann

    2013-07-01

    Full Text Available The equation of the propagation of light in an expanding Universe is derived based on the definition of comoving distances. A numerical method is proposed to solve this equation jointly with the Friedmann equation. As the equation of the propagation of light in an expanding Universe defines a horizon of the visible Universe, this puts a constraint on cosmological models in order to be consistent with an upper limit for redshifts observed from galaxies. This puzzle is challenging current expansionist cosmological models.

  16. 3D CFD analysis of a twin screw expander

    OpenAIRE

    Kovacevic, A.; S Rane

    2013-01-01

    Twin screw machines can be used as expanders for variety of applications. This paper describes how the performance of an oil free twin screw air expander of 3/5 lobe configuration was estimated by use of full 3D Computational Fluid Dynamics (CFD) applying a procedure similar to that used for screw compressors. The grid generator SCORG© was employed for pre-processing of the moving domains between the rotors while the stationary grids for the ports were derived from a commercial grid generator...

  17. Pre-expanded Intercostal Perforator Super-Thin Skin Flap.

    Science.gov (United States)

    Liao, Yunjun; Luo, Yong; Lu, Feng; Hyakusoku, Hiko; Gao, Jianhua; Jiang, Ping

    2017-01-01

    This article introduces pre-expanded super-thin intercostal perforator flaps, particularly the flap that has a perforator from the first to second intercostal spaces. The key techniques, advantages and disadvantages, and complications and management of this flap are described. At present, the thinnest possible flap is achieved by thinning the pre-expanded flap that has a perforator from the first to second intercostal spaces. It is used to reconstruct large defects on the face and neck, thus restoring function and cosmetic appearance. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Energy Emission by Quantum Systems in an Expanding FRW Metric

    CERN Document Server

    Sheehan, D P

    2004-01-01

    Bound quantum mechanical systems not expanding with the comoving frame of an expanding, flat FRW metric are found to release energy at a rate linearly proportional to the local Hubble constant ($H_{o}$) and the systems' binding energy ($E_{b}$); {\\em i.e.}, $\\dot{E} = H_{o} E_{b}$. Three exemplary quantum systems are examined. For systems with early cosmological condensation times | notably hadrons | time-integrated energy release could have been significant and could account for an appreciable fraction of the dark matter inventory.

  19. Competition with Dynamic Spectrum Leasing

    CERN Document Server

    Duan, Lingjie; Shou, Biying

    2010-01-01

    This paper presents a comprehensive analytical study of two competitive cognitive operators' spectrum leasing and pricing strategies, taking into account operators' heterogeneity in leasing costs and users' heterogeneity in transmission power and channel conditions. We model the interactions between operators and users as a three-stage dynamic game, where operators make simultaneous spectrum leasing and pricing decisions in Stages I and II, and users make purchase decisions in Stage III. Using backward induction, we are able to completely characterize the game's equilibria. We show that both operators make the equilibrium leasing and pricing decisions based on simple threshold policies. Moreover, two operators always choose the same equilibrium price despite their difference in leasing costs. Each user receives the same signal-to-noise-ratio (SNR) at the equilibrium, and the obtained payoff is linear in its transmission power and channel gain. We also compare the duopoly equilibrium with the coordinated case ...

  20. Image restoration using spectrum estimation

    Science.gov (United States)

    Na, Ki-Woon; Paik, Joon-Ki

    1994-09-01

    A stochastic approach to image restoration is proposed by using various spectrum estimation techniques. In order to estimate the original image from the knowledge of observed image, the minimum mean square error filter or Wiener filter is known to be optimum in the sense of minimizing the mean square error. The optimality of Wiener filter, however, holds only when the power spectra of the original image and noise are given in addition to the transfer function of the imaging system. In practice, the information of the original image is generally not available. In the present paper additive noise is assumed to be white with known variance and the Wiener filter is implemented using various estimation techniques for the original spectrum. The proposed method shows significant improvement over the conventional methods, such as the Wiener filter using constant signal-to-noise power ratio, particularly for images with low signal-to-noise ratio.

  1. Orthopositronium Decay Spectrum using NRQED

    CERN Document Server

    Ruiz-Femenia, P D

    2006-01-01

    As noticed in Ref.[1], the Ore-Powell's classical calculation of the o-Ps -> 3 gamma decay amplitude does not fulfill Low's theorem requirements for the low energy end of the photon spectrum. We reanalyze the implications of Low's theorem applied to this decay considering the interplay between the soft photon limit and the energy scales present in the e+e- system. For energetic photons, the spectrum agrees with the Ore-Powell result, but deviates from it when the photon energy is comparable to the positronium binding energy. In this region it is found that bound states effects are essential to reach agreement with Low's theorem and can be accounted for in the framework of non-relativistic QED.

  2. Rigidity spectrum of Forbush decrease

    Science.gov (United States)

    Sakakibara, S.; Munakata, K.; Nagashima, K.

    1985-01-01

    Using data from neutron monitors and muon telescopes at surface and underground stations, the average rigidity spectrum of Forbush decreases (Fds) during the period of 1978-1982 were obtained. Thirty eight Ed-events are classified into two groups Hard Fd and Soft Fd according to size of Fd at Sakashita station. It is found that a spectral form of fractional-power type (P to the-gamma sub 1 (P+P sub c) to the -gamma sub2) is more suitable for the present purpose than that of power-exponential type or of power type with an upper limiting rigidity. The best fitted spectrum of fractional-power type is expressed by gamma sub1 = 0.37, gamma sub2 = 0.89 and P subc = 10 GV for Hard Fd and gamma sub1 = 0.77, gamma sub2 = 1.02 and P sub c - 14GV for Soft Fd.

  3. Measurement of Hot Electron Spectrum

    Institute of Scientific and Technical Information of China (English)

    LIYe-jun; SHANYu-sheng; ZHANGHai-feng; ZHANGJi; WANGLei-jian; TANGXiu-zhang

    2003-01-01

    The hot electron spectrum was measured using 180°electron magnetic spectrometer through the irradiation of solid Cu target by an intense, near infrared(744 nm), P-polarized light, femtosecond (120 fs) laser pulse with free pre-pulse, and the intensity of laser is 1016 W/cm2 with 45° incidence. And the spectrometer was located in the direction of laser right reflection.

  4. Cooperative Distributed Sequential Spectrum Sensing

    CERN Document Server

    S, Jithin K; Gopalarathnam, Raghav

    2010-01-01

    We consider cooperative spectrum sensing for cognitive radios. We develop an energy efficient detector with low detection delay using sequential hypothesis testing. Sequential Probability Ratio Test (SPRT) is used at both the local nodes and the fusion center. We also analyse the performance of this algorithm and compare with the simulations. Modelling uncertainties in the distribution parameters are considered. Slow fading with and without perfect channel state information at the cognitive radios is taken into account.

  5. Commutators with Finite Spectrum

    Institute of Scientific and Technical Information of China (English)

    Nadia BOUDI

    2009-01-01

    The purpose of this paper is to study derivations d, d' defined on a Banach algebra A such that the spectrum σ([dx, d'x]) is finite for all x ∈ A. In particular we show that if the algebra is semisimple, then there exists an element a in the socle of A such that [d, d'] is the inner derivation implemented by a.

  6. Hydrodynamics of the Dirac spectrum

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Yizhuang, E-mail: yizhuang.liu@stonybrook.edu [Department of Physics and Astronomy, Stony Brook University, Stony Brook, NY 11794-3800 (United States); Warchoł, Piotr, E-mail: piotr.warchol@uj.edu.pl [M. Smoluchowski Institute of Physics, Jagiellonian University, PL-30348 Krakow (Poland); Zahed, Ismail, E-mail: ismail.zahed@stonybrook.edu [Department of Physics and Astronomy, Stony Brook University, Stony Brook, NY 11794-3800 (United States)

    2016-02-10

    We discuss a hydrodynamical description of the eigenvalues of the Dirac spectrum in even dimensions in the vacuum and in the large N (volume) limit. The linearized hydrodynamics supports sound waves. The hydrodynamical relaxation of the eigenvalues is captured by a hydrodynamical (tunneling) minimum configuration which follows from a pertinent form of Euler equation. The relaxation from a phase of unbroken chiral symmetry to a phase of broken chiral symmetry occurs over a time set by the speed of sound.

  7. ACE spectrum of LDPC codes

    Directory of Open Access Journals (Sweden)

    Vukobratović Dejan

    2006-01-01

    Full Text Available Construction of short-length LDPC codes with good, both waterfall and error-floor, behavior is still an attractive research problem. Recently proposed construction algorithms in this field are based on remarkably simple ideas, but yet, their effectiveness can still be questioned. In this paper we investigate a novel measure of goodness of a given LDPC code namely its ACE spectrum, based on a previously introduced ACE metrics associated with each cycle in LDPC code graph.

  8. Baryon spectrum and chiral dynamics

    CERN Document Server

    Glozman, L Ya

    1995-01-01

    New results on baryon structure and spectrum developed in collaboration with Dan Riska [1-4] are reported. The main idea is that beyond the chiral symmetry spontaneous breaking scale light and strange baryons should be considered as systems of three constituent quarks with an effective confining interaction and a chiral interaction that is mediated by the octet of Goldstone bosons (pseudoscalar mesons) between the constituent quarks.

  9. Physically Protected Spread Spectrum Communications

    Science.gov (United States)

    2016-04-14

    for the FH system in those days was because of implementation issues, such as a frequency -ringing problem , which occurred whenever a frequency was...PS-TR-2016-0046 HAS BEEN REVIEWED AND IS APPROVED FOR PUBLICATION IN ACCORDANCE WITH ASSIGNED DISTRIBUTION STATEMENT. KHANH PHAM PAUL D. LEVAN...keying (MPSK) and M-ary quadrature amplitude-shift keying (MQAM) for a slow frequency hopping (FH) spread spectrum (SS) system because, recently

  10. Voice Collection under Different Spectrum

    Directory of Open Access Journals (Sweden)

    Min Li

    2013-05-01

    Full Text Available According to the short-time Fourier transform theory and principle of digital filtering, this paper established a mathematical model called collection of voice signal collection at different spectrum. The voice signal was a non-stationary process, while the standard Fourier transform only applied to the periodic signal, transient signals or stationary random signal. Therefore, the standard Fourier transform could not be directly used for the speech signal. By controlling the input different types and parameters, this paper analyzed the collected original voice signal spectrum with the use of MATLAB software platform. At the same time, it realized the extraction, recording and playback of the speech signal at different frequencies. Therefore, the waveforms could be displayed obviously on the graphic user interface and voice effect could be more clearly. Meanwhile, the result was verified by the hardware platforms, which consisted of TMS320VC5509A [1] chip and TLV320AIC23 voice chip. The results showed that the extraction of voice signal under different spectrum model was scientific, rational and effective.

  11. Aortic annulus eccentricity before and after transcatheter aortic valve implantation: Comparison of balloon-expandable and self-expanding prostheses

    Energy Technology Data Exchange (ETDEWEB)

    Schuhbaeck, Annika, E-mail: annika.schuhbaeck@uk-erlangen.de [Department of Cardiology, University of Erlangen, Erlangen (Germany); Weingartner, Christina [Department of Cardiology, University of Gießen, Giessen (Germany); Arnold, Martin; Schmid, Jasmin; Pflederer, Tobias; Marwan, Mohamed [Department of Cardiology, University of Erlangen, Erlangen (Germany); Rixe, Johannes; Nef, Holger [Department of Cardiology, University of Gießen, Giessen (Germany); Schneider, Christian [Department of Radiology, University of Gießen, Giessen (Germany); Lell, Michael; Uder, Michael [Department of Radiology, University of Erlangen, Erlangen (Germany); Ensminger, Stephan [Department of Thoracic and Cardiovascular Surgery, Heart and Diabetes Center NRW, Ruhr-University Bochum, Bad Oeynhausen (Germany); Feyrer, Richard; Weyand, Michael [Department of Cardiothoracic Surgery, University of Erlangen, Erlangen (Germany); Achenbach, Stephan [Department of Cardiology, University of Erlangen, Erlangen (Germany)

    2015-07-15

    Highlights: • Post-implant geometry of catheter-based aortic valve prostheses is influenced by aortic valve calcification. • Balloon-expandable prostheses are more circular as compared to self-expanding prostheses. • The impact of post-implant geometry on valve function needs to be investigated. - Abstract: Introduction: The geometry of the aortic annulus and implanted transcatheter aortic valve prosthesis might influence valve function. We investigated the influence of valve type and aortic valve calcification on post-implant geometry of catheter-based aortic valve prostheses. Methods: Eighty consecutive patients with severe aortic valve stenosis (mean age 82 ± 6 years) underwent computed tomography before and after TAVI. Aortic annulus diameters were determined. Influence of prosthesis type and degree of aortic valve calcification on post-implant eccentricity were analysed. Results: Aortic annulus eccentricity was reduced in patients after TAVI (0.21 ± 0.06 vs. 0.08 ± 0.06, p < 0.0001). Post-TAVI eccentricity was significantly lower in 65 patients following implantation of a balloon-expandable prosthesis as compared to 15 patients who received a self-expanding prosthesis (0.06 ± 0.05 vs. 0.15 ± 0.07, p < 0.0001), even though the extent of aortic valve calcification was not different. After TAVI, patients with a higher calcium amount retained a significantly higher eccentricity compared to patients with lower amounts of calcium. Conclusions: Patients undergoing TAVI with a balloon-expandable prosthesis show a more circular shape of the implanted prosthesis as compared to patients with a self-expanding prosthesis. Eccentricity of the deployed prosthesis is affected by the extent of aortic valve calcification.

  12. Hadron Structure and Spectrum from the Lattice

    CERN Document Server

    Lang, C B

    2015-01-01

    Lattice calculations for hadrons are now entering the domain of resonances and scattering, necessitating a better understanding of the observed discrete energy spectrum. This is a reviewing survey about recent lattice QCD results, with some emphasis on spectrum and scattering.

  13. Neutron spectrum unfolding: Pt. 1; Theoretical

    Energy Technology Data Exchange (ETDEWEB)

    Matiullah (Centre for Nuclear Studies, Nilore, Islamabad (Pakistan)); Wiyaja, D.S. (PPTN - BATAN, Bandung (Indonesia)); Berzonis, M.A.; Bondars, H.; Lapenas, A.A. (Latvijskij Gosudarstvennyj Univ., Riga (USSR)); Kudo, K. (Electrotechnical Lab., Tsukuba, Ibaraki (Japan)); Majeed, A.; Durrani, S.A. (Birimingham Univ. (United Kingdom). School of Physics and Space Research)

    1991-01-01

    The use of the latest PC version of the computer code SAIPS in neutron spectrum unfolding is described. Guidelines for extending the use of SAIPS to unfold the spectrum from a CR-39-based spectrometer are presented. (author).

  14. Smart Radio Spectrum Management for Cognitive Radio

    Directory of Open Access Journals (Sweden)

    Partha Pratim Bhattacharya

    2011-08-01

    Full Text Available Today’s wireless networks are characterized by fixed spectrum assignment policy. The limited availablespectrum and the inefficiency in the spectrum usage necessitate a new communication paradigm toexploit the existing wireless spectrum opportunistically. Cognitive radio is a paradigm for wirelesscommunication in which either a network or a wireless node changes its transmission or receptionparameters to communicate efficiently avoiding interference with licensed or unlicensed users. It cancapture best available spectrum to meet user communication requirements (spectrum management. Inthis work, a fuzzy logic based system for spectrum management is proposed where the radio can shareunused spectrum depending on parameters like distance, signal strength, node velocity and availabilityof unused spectrum. The system is simulated and is found to give satisfactory results.

  15. Hubble Diagram Test of Expanding and Static Cosmological Models: The Case for a Slowly Expanding Flat Universe

    Directory of Open Access Journals (Sweden)

    Laszlo A. Marosi

    2013-01-01

    Full Text Available We present a new redshift (RS versus photon travel time ( test including 171 supernovae RS data points. We extended the Hubble diagram to a range of z = 0,0141–8.1 in the hope that at high RSs, the fitting of the calculated RS/ diagrams to the observed RS data would, as predicted by different cosmological models, set constraints on alternative cosmological models. The Lambda cold dark matter (ΛCDM, the static universe model, and the case for a slowly expanding flat universe (SEU are considered. We show that on the basis of the Hubble diagram test, the static and the slowly expanding models are favored.

  16. EXPANDED PERLITE, EXPANDED VERMICULITE AND MICROSPHERES AS FILLERS IN NEW GENERATION PAPER PULP MIXTURES USED FOR CONTACT WITH LIQUID METAL

    OpenAIRE

    Zbigniew Zawieja; Jacek Sawicki

    2015-01-01

    Liquid metal when filling sand casting mould while pouring it out from ladle at the first moment comes across the sprue/gate system of the mould the purpose of which is to transfer liquid metal and feed the mould recess. The materials presently used for the elements of the sprue/gate systems are based on ceramics or the mixtures based on paper pulp. In this study the use of alternative mineral additions such as expanded perlite, expanded vermiculite, and microspheres as the fillers to paper p...

  17. An Expanded Model of Faculty Vitality in Academic Medicine

    Science.gov (United States)

    Dankoski, Mary E.; Palmer, Megan M.; Laird, Thomas F. Nelson; Ribera, Amy K.; Bogdewic, Stephen P.

    2012-01-01

    Many faculty in today's academic medical centers face high levels of stress and low career satisfaction. Understanding faculty vitality is critically important for the health of our academic medical centers, yet the concept is ill-defined and lacking a comprehensive model. Expanding on previous research that examines vital faculty in higher…

  18. 2010 Impacts: The Expanded Food and Nutrition Education Program (EFNEP)

    Science.gov (United States)

    National Institute of Food and Agriculture, 2011

    2011-01-01

    Since 1969, the Expanded Food and Nutrition Education Program (EFNEP) has improved the diets and food-related behaviors of program participants. Each year EFNEP enrolls more than half a million new program participants. In 2010, EFNEP reached 137,814 adults and 463,530 youth directly and nearly 400,000 family members indirectly. This paper…

  19. The Expanded Food and Nutrition Education Program (EFNEP)

    Science.gov (United States)

    National Institute of Food and Agriculture, 2010

    2010-01-01

    Obesity, poor health, and limited physical activity are major health concerns. The Expanded Food and Nutrition Education Program (EFNEP) improves the health and well-being of limited resource families and youth. Additionally, EFNEP leads to public savings. Research shows that better health is associated with reduced health care costs, less…

  20. Expanded indications for combo ceramic onlays/inlays.

    Science.gov (United States)

    Joffe, Eugene

    2008-04-01

    The ability to use conservative ceramic restorations may be limited because of the location and placement of the gingival margin while preparing the tooth for an inlay/onlay. The following technique, which uses a combination of modified preparation and special processing of a restoration combining gold and ceramic, may actually help expand the conservative treatment modality.

  1. Expanding the taxonomy of the diagnostic criteria for temporomandibular disorders

    NARCIS (Netherlands)

    Peck, C.C.; Goulet, J-P; Lobbezoo, F.; Schiffman, E.L.; Alstergren, P.; Anderson, G.C.; De Leeuw, R.; Jensen, R.; Michelotti, A.; Ohrbach, R.; Petersson, A.; List, T.

    2014-01-01

    There is a need to expand the current temporomandibular disorders' (TMDs) classification to include less common but clinically important disorders. The immediate aim was to develop a consensus-based classification system and associated diagnostic criteria that have clinical and research utility for

  2. Dynamical 3-Space: Black Holes in an Expanding Universe

    Directory of Open Access Journals (Sweden)

    Rothall D. P.

    2013-10-01

    Full Text Available Black holes are usually studied without including effects of the expanding universe. However in some recent studies black holes have been embedded in an expanding universe, in order to determine the interplay, if any, of these two dynamical processes. Dynamical 3-space theory contains time independent solutions for black holes, which are spatial in-flows, and separately the time dependent Hubble expansion. This theory has explained numerous puzzles in observational astrophysics and contains 3 constants; G, - which from experimental data turns out to be the fine structure constant, and - which is a small but nonzero distance, possibly a Planck-type length. The Hubble expansion in the dynamical 3-space theory cannot be “switched o”, forcing the study, first, of isolated black holes coexisting with the expanding universe. It is shown that a time dependent black hole and expanding universe solution exists. The nature and implications of these solutions are discussed as they evolve over time. A dynamical network of black holes and induced linking cosmic filaments forming bubble structures is discussed, as a consequence of dynamical 3-space undergoing a dynamical breakdown of homogeneity and isotropy, even in the absence of baryonic matter.

  3. Expanding the Spanish Classroom: The "Art" in Liberal Arts

    Science.gov (United States)

    Díaz, Erin M.

    2016-01-01

    Supplementing the foreign language curriculum with the incorporation of art museum visits has benefits for students, faculty, the campus art gallery, and the institution. Such a collaborative program serves to expand the classroom and complement instruction by providing learners with a new space to engage in authentic practice in the target…

  4. Expanding Schooling Opportunities for 4-Year-Olds

    Science.gov (United States)

    Leuven, Edwin; Lindahl, Mikael; Oosterbeek, Hessel; Webbink, Dinand

    2010-01-01

    We use a novel quasi-experimental strategy to estimate the effect of expanding early schooling enrollment possibilities on early achievement. It exploits two features of the school system in The Netherlands. The first is rolling admissions; children are allowed to start school immediately after their 4th birthday instead of at the beginning of the…

  5. Expanding the Role of Connectionism in SLA Theory

    Science.gov (United States)

    Language Learning, 2013

    2013-01-01

    In this article, I explore how connectionism might expand its role in second language acquisition (SLA) theory by showing how some symbolic models of bilingual and second language lexical memory can be reduced to a biologically realistic (i.e., neurally plausible) connectionist model. This integration or hybridization of the two models follows the…

  6. Validation of the Chinese Expanded Euthanasia Attitude Scale

    Science.gov (United States)

    Chong, Alice Ming-Lin; Fok, Shiu-Yeu

    2013-01-01

    This article reports the validation of the Chinese version of an expanded 31-item Euthanasia Attitude Scale. A 4-stage validation process included a pilot survey of 119 college students and a randomized household survey with 618 adults in Hong Kong. Confirmatory factor analysis confirmed a 4-factor structure of the scale, which can therefore be…

  7. Validation of the Chinese Expanded Euthanasia Attitude Scale

    Science.gov (United States)

    Chong, Alice Ming-Lin; Fok, Shiu-Yeu

    2013-01-01

    This article reports the validation of the Chinese version of an expanded 31-item Euthanasia Attitude Scale. A 4-stage validation process included a pilot survey of 119 college students and a randomized household survey with 618 adults in Hong Kong. Confirmatory factor analysis confirmed a 4-factor structure of the scale, which can therefore be…

  8. Expanding Gerontology Enrollments: Successful Results of an Innovative Outreach Program

    Science.gov (United States)

    Reynolds, Sandra L.; Haley, William E.; Hyer, Kathryn

    2007-01-01

    As state budget allocations for higher education decrease, "specialty" programs such as gerontology must continually demonstrate their productivity. State and private universities increasingly rely on student credit hours (SCH) or tuition generated, which is making it difficult for many gerontology programs to expand. The School of Aging Studies…

  9. THE HAMILTONIAN STRUCTURE OF TWO INTEGRABLE EXPANDING MODELS

    Institute of Scientific and Technical Information of China (English)

    Liu Bin; Dong Huanhe; Li Zhu

    2007-01-01

    In this paper,an extended loop algebra is constructed from which an isospectral problem established.It follows that the integrable couplings of the Tu hierarchy and M-AKNS-KN hierarchy are obtained.and their Hamilton structures are presented by the quadratic-form identity.Moreover,we guarantee that the expanding model we obtained are also Liouville integrable.

  10. Enrichment in Massachusetts Expanded Learning Time (ELT) Schools. Issue Brief

    Science.gov (United States)

    Caven, Meghan; Checkoway, Amy; Gamse, Beth; Luck, Rachel; Wu, Sally

    2012-01-01

    This brief highlights key information about enrichment activities, which represent one of the main components of the Massachusetts Expanded Learning Time (ELT) initiative. Over time, the ELT initiative has supported over two dozen schools across the Commonwealth. A comprehensive evaluation of the ELT initiative found that implementation of the…

  11. Collaborative Planning in Massachusetts Expanded Learning Time (ELT). Issue Brief

    Science.gov (United States)

    Caven, Meghan; Checkoway, Amy; Gamse, Beth

    2012-01-01

    The Massachusetts Expanded Learning Time (ELT) initiative is designed to improve academic outcomes by increasing the amount of learning time for students. Schools are expected to provide opportunities to teachers for collaborative planning and professional development as well as add time to the school day or year for instructional and enrichment…

  12. Expanding Gerontology Enrollments: Successful Results of an Innovative Outreach Program

    Science.gov (United States)

    Reynolds, Sandra L.; Haley, William E.; Hyer, Kathryn

    2007-01-01

    As state budget allocations for higher education decrease, "specialty" programs such as gerontology must continually demonstrate their productivity. State and private universities increasingly rely on student credit hours (SCH) or tuition generated, which is making it difficult for many gerontology programs to expand. The School of Aging Studies…

  13. Fire Resistance of Concrete Constructions with Expanded Clay Aggregate

    DEFF Research Database (Denmark)

    Hertz, Kristian Dahl

    2003-01-01

    The paper describes briefly a series of full-scale tests made as documentation for extending the field of application of the reduced cross section method from the structural codes to constructions based on expanded clay aggregate. As a special result the tests verify that slabs with a short...

  14. Role of self expandable stents in management of colorectalcancers

    Institute of Scientific and Technical Information of China (English)

    2016-01-01

    Acute malignant colorectal obstruction is a complicationof colorectal cancer that can occur in 7%-29% of patients. Self-expanding metallic stent placement formalignant colorectal obstruction has gained popularity asa safe and effective procedure for relieving obstruction.This technique can be used in the palliation of malignantcolorectal obstruction, as a bridge to elective surgery forresectable colorectal cancers, palliation of extracolonicmalignant obstruction, and for nonmalignant etiologiessuch as anastomotic strictures, Crohn's disease, radiationtherapy, and diverticular diseases. Self-expandingmetallic stent has its own advantages and disadvantagesover the surgery in these indications. During the insertionof the self-expanding metallic stent, and in the followup,short term and long term morbidities should be keptin mind. The most important complications of the stentsare perforation, stent obstruction, stent migration, andbleeding. Additionally, given the high risk of perforation,if a patient is treated or being consideredfortreatmentwithantiangiogenic agents such as bevacizumab, it isnot recommended to use self-expanding metallic stentas a palliative treatment for obstruction. Therefore, thereis a need for careful clinical evaluation for each patientwho is a candidate for this procedure. The purpose ofthis review was to evaluate self-expanding metallic stentin the management of the obstruction of the colon dueto the colorectal and extracolonic obstruction.

  15. Expanding the Role of Connectionism in SLA Theory

    Science.gov (United States)

    Language Learning, 2013

    2013-01-01

    In this article, I explore how connectionism might expand its role in second language acquisition (SLA) theory by showing how some symbolic models of bilingual and second language lexical memory can be reduced to a biologically realistic (i.e., neurally plausible) connectionist model. This integration or hybridization of the two models follows the…

  16. Expandable bioresorbable endovascular stent. I. Fabrication and properties.

    Science.gov (United States)

    Su, Shih-Horng; Chao, Robert Y N; Landau, Charles L; Nelson, Kevin D; Timmons, Richard B; Meidell, Robert S; Eberhart, Robert C

    2003-06-01

    A bioresorbable, expandable poly(L-lactic acid) stent has been designed, based on a linear, continuous coil array principle, by which multiple furled lobes convert to a single lobe upon balloon expansion, without heating. Stent strength and compliance are sufficient to permit deployment by a conventional balloon angioplasty catheter. Several multiple lobe configurations were investigated, with expansion ratios ranging from 1.4 to 1.9 and expanded diameters ranging from 2.3 to 4.7 mm. Compression resistance of the expanded stent is dependent on fiber coil density and fiber ply. A range sufficient for endovascular service was obtained, with less than 4% elastic recoil in six day saline incubation studies. Surface plasma treatment with di(ethylene glycol) vinyl ether significantly reduced platelet adhesion in a 1 h porcine arteriovenous shunt model. Patency was maintained in one week implant studies in the porcine common femoral artery. However, a strong inflammatory response, and significant reduction of the vascular lumen were observed following two weeks implantation. The design principles and fabrication techniques for this bioresorbable stent are sufficiently versatile that a broad range of applications can be addressed. Much work remains to be done, including long-term evaluation of the inflammatory response, and of polymer degradation. The results of this study demonstrate the feasibility of expandable biodegradable stent design and deployment by conventional means.

  17. Interpreting and Expanding Confucius' Golden Mean through Neutrosophic Tetrad

    Directory of Open Access Journals (Sweden)

    Fu Yuhua

    2016-03-01

    Full Text Available Neutrosophy is a new branch of philosophy that studies the origin, nature, and scope of neutralities, as well as their interactions with different ideational spectra. There are many similarities between The Golden Mean and Neutrosophy. Chinese and international scholars need to toil towards expanding and developing The Golden Mean, towards its "modernization" and "globalization".

  18. Expanding the Vision of Self: Why the Arts Matter

    Science.gov (United States)

    Nathan, Linda F.

    2013-01-01

    In this reflective essay, Linda F. Nathan, the founding headmaster of the Boston Arts Academy and currently the executive director of Center for Arts in Education at Boston Arts Academy, shares a story about how one student, Ronald, expands his vision of self through his engagement with the arts. In presenting this reflection on Ronald's…

  19. Diaspora, Migration, and Globalization: Expanding the Discourse of Adult Education

    Science.gov (United States)

    Alfred, Mary V.

    2015-01-01

    This article explores how notions of diaspora, migration, and globalization intersect to inform identities and social realities of those who leave their homeland and resettle in other nations. It calls for expanding the discourse of adult education to incorporate critical studies of the diaspora to make visible the inequality and imbalance of…

  20. Time Impact of Expanded Process Control Procedures (EPCP)

    Science.gov (United States)

    2014-06-01

    than the Organizational maintenance. Depot maintenance occurs in the manufacturer’s specialized shops . This type of work is generally the most...EXPANDED PROCESS CONTROL PROCEDURES (EPCP) by Jeremy C. Joseph June 2014 Thesis Advisor: Gary O. Langford Second Reader: Diana I. Angelis...

  1. Targeting China's Newly Rich Western Luxury Brands to Expand

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    @@ Western fashion brands have been considering China as a market for cheap raw materials and labor for several centuries.However,in recent months,China's booming economy has attracted the fashion industry.Many high-profit fashion brands said they intend to expand business in China.

  2. Expanding Hipolito-Delgado and Lee's Empowerment Theory: A Response

    Science.gov (United States)

    Zalaquett, Carlos; D'Andrea, Michael

    2007-01-01

    Hipolito-Delgado and Lee's article entitled "Empowerment Theory for the Professional School Counselor: A Manifesto for What Really Matters" represents an important contribution to the school counseling literature for two reasons: (1) It helps to expand a growing knowledge base related to the work school counselors can do to foster the…

  3. Expanding Distribution of Lethal Amphibian Fungus Batrachochytrium salamandrivorans in Europe.

    Science.gov (United States)

    Spitzen-van der Sluijs, Annemarieke; Martel, An; Asselberghs, Johan; Bales, Emma K; Beukema, Wouter; Bletz, Molly C; Dalbeck, Lutz; Goverse, Edo; Kerres, Alexander; Kinet, Thierry; Kirst, Kai; Laudelout, Arnaud; Marin da Fonte, Luis F; Nöllert, Andreas; Ohlhoff, Dagmar; Sabino-Pinto, Joana; Schmidt, Benedikt R; Speybroeck, Jeroen; Spikmans, Frank; Steinfartz, Sebastian; Veith, Michael; Vences, Miguel; Wagner, Norman; Pasmans, Frank; Lötters, Stefan

    2016-07-01

    Emerging fungal diseases can drive amphibian species to local extinction. During 2010-2016, we examined 1,921 urodeles in 3 European countries. Presence of the chytrid fungus Batrachochytrium salamandrivorans at new locations and in urodeles of different species expands the known geographic and host range of the fungus and underpins its imminent threat to biodiversity.

  4. Expanding the mirror : vicarious activity for actions, emotions, and sensations

    NARCIS (Netherlands)

    Keysers, Christian; Gazzola, Valeria

    2009-01-01

    We often empathically share the states of others. The discovery of 'mirror neurons' suggested a neural mechanism for monkeys to share the actions of others. Here we expand this view by showing that mirror neurons for actions not only exist in the premotor cortex or in monkeys and that vicarious acti

  5. Lattice expansion of carbon-stabilized expanded austenite

    DEFF Research Database (Denmark)

    Hummelshøj, Thomas Strabo; Christiansen, Thomas; Somers, Marcel A. J.

    2010-01-01

    The lattice parameter of expanded austenite was determined as a function of the content of interstitially dissolved carbon in homogeneous, carburized thin stainless steel foils. For the first time this expansion of the face-centered cubic lattice is determined on unstrained austenite. It is found...

  6. Crowd Around: Expanding Your Donor Pool with Crowdfunding

    Science.gov (United States)

    Jarrell, Andrea

    2013-01-01

    At most institutions, annual fund-giving is down. Crowdfunding sites allow people with a great idea or worthy cause to bypass traditional funding methods and take their case directly to web-savvy investors and donors. This article describes how higher education institutions are expanding their donor pool through such crowdfunding sites as USEED,…

  7. Response to "Expanding Access to Learning with Mobile Digital Devices"

    Science.gov (United States)

    Vanek, Jen

    2017-01-01

    In his article "Expanding Access to Learning with Mobile Digital Devices" (EJ1150752), Jeff Carter recommended a balanced perspective when measuring the potential of mobile learning to redefine teaching and learning for adults with basic skills needs. In response to Carter's article, the author makes some recommendations that she thinks…

  8. How to expand the corpus of spoken English

    Institute of Scientific and Technical Information of China (English)

    张光华

    2015-01-01

    With the speeding up of economic globalization, English as a global language status is becoming more and more manifest.This article from the spoken corpus concept is introduced, combined with the students' oral English learning methods, guide the students in learning spoken English in different ways to find the effective way to expand the spoken language corpora.

  9. Expanding the Black Woman's Horizon: Picking From a Higher Bush ...

    African Journals Online (AJOL)

    Expanding the Black Woman's Horizon: Picking From a Higher Bush Motif in Zora ... While bringing up Janie, Nanny overprotects her and shields her from the ... In her quest, Janie discovers that it is not comfort or money that brings about love ...

  10. Expanding the taxonomy of the diagnostic criteria for temporomandibular disorders.

    Science.gov (United States)

    Peck, C C; Goulet, J-P; Lobbezoo, F; Schiffman, E L; Alstergren, P; Anderson, G C; de Leeuw, R; Jensen, R; Michelotti, A; Ohrbach, R; Petersson, A; List, T

    2014-01-01

    There is a need to expand the current temporomandibular disorders' (TMDs) classification to include less common but clinically important disorders. The immediate aim was to develop a consensus-based classification system and associated diagnostic criteria that have clinical and research utility for less common TMDs. The long-term aim was to establish a foundation, vis-à-vis this classification system, that will stimulate data collection, validity testing and further criteria refinement. A working group [members of the International RDC/TMD Consortium Network of the International Association for Dental Research (IADR), members of the Orofacial Pain Special Interest Group (SIG) of the International Association for the Study of Pain (IASP), and members from other professional societies] reviewed disorders for inclusion based on clinical significance, the availability of plausible diagnostic criteria and the ability to operationalise and study the criteria. The disorders were derived from the literature when possible and based on expert opinion as necessary. The expanded TMDs taxonomy was presented for feedback at international meetings. Of 56 disorders considered, 37 were included in the expanded taxonomy and were placed into the following four categories: temporomandibular joint disorders, masticatory muscle disorders, headache disorders and disorders affecting associated structures. Those excluded were extremely uncommon, lacking operationalised diagnostic criteria, not clearly related to TMDs, or not sufficiently distinct from disorders already included within the taxonomy. The expanded TMDs taxonomy offers an integrated approach to clinical diagnosis and provides a framework for further research to operationalise and test the proposed taxonomy and diagnostic criteria.

  11. Reflections on Using Blogs to Expand In-Class Discussion

    Science.gov (United States)

    Wang, Shiang-Kwei; Hsua, Hui-Yin

    2008-01-01

    Originally, blogs served as personal journaling tools. Recently, blogs have facilitated the formation of online communities and have thus expanded to more extensive uses in education. Little has been learned about using blogs as a collaboration tool in educational contexts. In this article, the authors share their experiences with using blogs to…

  12. CSU Standard for the CLSI Expanded Title Record.

    Science.gov (United States)

    Anderson, Karen; And Others

    The revised system specifications described in this document were adopted by the 19 California State University and College (CSUC) libraries for a second test version of the CLSI "Expanded Title Record" public access online catalog at California State University at Chico. Should this pilot demonstration of the data format prove…

  13. CSUC Standard for the CLSI Expanded Title Record.

    Science.gov (United States)

    Case, Barbara; And Others

    The system specifications described have been adopted by the 19 California State University and Colleges Libraries for use with the CL Systems "Expanded Title Record" format. It is anticipated that these specifications will be tested at California State University, Chico, beginning midyear 1981. Should this pilot demonstration prove…

  14. Diaspora, Migration, and Globalization: Expanding the Discourse of Adult Education

    Science.gov (United States)

    Alfred, Mary V.

    2015-01-01

    This article explores how notions of diaspora, migration, and globalization intersect to inform identities and social realities of those who leave their homeland and resettle in other nations. It calls for expanding the discourse of adult education to incorporate critical studies of the diaspora to make visible the inequality and imbalance of…

  15. New expanded bed adsorbents for the recovery of DNA

    DEFF Research Database (Denmark)

    Theodossiou, Irini; Olander, M. A.; Sondergaard, M.;

    2000-01-01

    A 20-40 mum pellicular high density (similar to3.7 g cm(-3)) expanded bed material has been designed for the capture of DNA and other large macromolecules. Anion exchangers fashioned out of these supports exhibited dramatically enhanced DNA binding capacities over commercial anion exchange...

  16. How to expand the corpus of spoken English

    Institute of Scientific and Technical Information of China (English)

    张光华

    2015-01-01

    With the speeding up of economic globalization, English as a global language status is becoming more and more manifest.This article from the spoken corpus concept is introduced, combined with the students’ oral English learning methods, guide the students in learning spoken English in different ways to find the effective way to expand the spoken language corpora.

  17. Expanding the Vision of Self: Why the Arts Matter

    Science.gov (United States)

    Nathan, Linda F.

    2013-01-01

    In this reflective essay, Linda F. Nathan, the founding headmaster of the Boston Arts Academy and currently the executive director of Center for Arts in Education at Boston Arts Academy, shares a story about how one student, Ronald, expands his vision of self through his engagement with the arts. In presenting this reflection on Ronald's…

  18. Studies of an expanded trinucleotide repeat in transgenic mice

    Energy Technology Data Exchange (ETDEWEB)

    Bingham, P.; Wang, S.; Merry, D. [Univ. of Pennsylvania, Philadelphia, PA (United States)

    1994-09-01

    Spinal and bulbar muscular atrophy (SBMA) is a progressive motor neuron disease caused by expansion of a trinucleotide repeat in the androgen receptor gene (AR{sup exp}). AR{sup exp} repeats expand further or contract in approximately 25% of transmissions. Analogous {open_quotes}dynamic mutations{close_quotes} have been reported in other expanded trinucleotide repeat disorders. We have been developing a mouse model of this disease using a transgenic approach. Expression of the SBMA AR was documented in transgenic mice with an inducible promoter. No phenotypic effects of transgene expression were observed. We have extended our previous results on stability of the expanded trinucleotide repeat in transgenic mice in two lines carrying AR{sup exp}. Tail DNA was amplified by PCR using primers spanning the repeat on 60 AR{sup exp} transgenic mice from four different transgenic lines. Migration of the PCR product through an acrylamide gel showed no change of the 45 CAG repeat length in any progeny. Similarly, PCR products from 23 normal repeat transgenics showed no change from the repeat length of the original construct. Unlike the disease allele in humans, the expanded repeat AR cDNA in transgenic mice showed no change in repeat length with transmission. The relative stability of CAG repeats seen in the transgenic mice may indicate either differences in the fidelity of replicative enzymes, or differences in error identification and repair between mice and humans. Integration site or structural properties of the transgene itself might also play a role.

  19. Educated Japanese English: Expanding Oral/Aural Core Vocabulary

    Science.gov (United States)

    D'Angelo, James F.

    2005-01-01

    Having established the world's first undergraduate college of world Englishes, and in an "Expanding-Circle" setting, we have created the pilot environment for a new type of ELT curriculum. We must address the creation of a curriculum that is pervasively informed by the philosophy of world Englishes at both a macro and micro level. Certain…

  20. CSUC Standard for the CLSI Expanded Title Record.

    Science.gov (United States)

    Case, Barbara; And Others

    The system specifications described have been adopted by the 19 California State University and Colleges Libraries for use with the CL Systems "Expanded Title Record" format. It is anticipated that these specifications will be tested at California State University, Chico, beginning midyear 1981. Should this pilot demonstration prove…