Music lessons are associated with increased verbal memory in individuals with Williams syndrome.

Science.gov (United States)

Dunning, Brittany A; Martens, Marilee A; Jungers, Melissa K

2014-11-16

Williams syndrome (WS) is a genetic disorder characterized by intellectual delay and an affinity for music. It has been previously shown that familiar music can enhance verbal memory in individuals with WS who have had music training. There is also evidence that unfamiliar, or novel, music may also improve cognitive recall. This study was designed to examine if a novel melody could also enhance verbal memory in individuals with WS, and to more fully characterize music training in this population. We presented spoken or sung sentences that described an animal and its group name to 44 individuals with WS, and then tested their immediate and delayed memory using both recall and multiple choice formats. Those with formal music training (average duration of training 4½ years) scored significantly higher on both the spoken and sung recall items, as well as on the spoken multiple choice items, than those with no music training. Music therapy, music enjoyment, age, and Verbal IQ did not impact performance on the memory tasks. These findings provide further evidence that formal music lessons may impact the neurological pathways associated with verbal memory in individuals with WS, consistent with findings in typically developing individuals. Copyright © 2014 Elsevier Ltd. All rights reserved.

Global and local music perception in children with Williams syndrome.

Science.gov (United States)

Deruelle, Christine; Schön, Daniele; Rondan, Cécilie; Mancini, Josette

2005-04-25

Musical processing can be decomposed into the appreciation of global and local elements. This global/local dissociation was investigated with the processing of contour-violated and interval-violated melodies. Performance of a group of 16 children with Williams syndrome and a group of 16 control children were compared in a same-different task. Control participants were more accurate in detecting differences in the contour-violated than in the interval-violated condition while Williams syndrome individuals performed equally well in both conditions. This finding suggests that global precedence may occur at an early perceptual stage in normally developing children. In contrast, no such global precedence is observed in the Williams syndrome population. These data are discussed in the context of atypical cognitive profiles of individuals with Williams syndrome.

Mailability v. the Crusader: Williams v. O'Brien.

Science.gov (United States)

Simmons, Charles E.

The issues of prior restraint and press censorship are examined in this paper, which focuses on the 1970 Williams v. O'Brien court case. The paper discusses the litigation, in which Robert F. Williams, as an American citizen living in Peking, China, sued the United States Postmaster General over the banning of the May 1967 issue of "The…

Williams syndrome

Science.gov (United States)

Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. It may be passed down in families. ... history of the condition. However, people with Williams syndrome have a 50% chance of passing the disorder ...

Brief Report: Major Depressive Disorder with Psychotic Features in Williams Syndrome--A Case Series

Science.gov (United States)

Valdes, Francisca; Keary, Christopher J.; Mullett, Jennifer E.; Palumbo, Michelle L.; Waxler, Jessica L.; Pober, Barbara R.; McDougle, Christopher J.

2018-01-01

Descriptions of individuals with Williams syndrome (WS) and co-morbid major depressive disorder (MDD) with psychotic features have not appeared in the literature. In addition to reviewing previous reports of psychotic symptoms in persons with WS, this paper introduces clinical histories and therapeutic management strategies for three previously…

Skin findings in Williams syndrome.

Science.gov (United States)

Kozel, Beth A; Bayliss, Susan J; Berk, David R; Waxler, Jessica L; Knutsen, Russell H; Danback, Joshua R; Pober, Barbara R

2014-09-01

Previous examination in a small number of individuals with Williams syndrome (also referred to as Williams-Beuren syndrome) has shown subtly softer skin and reduced deposition of elastin, an elastic matrix protein important in tissue recoil. No quantitative information about skin elasticity in individuals with Williams syndrome is available; nor has there been a complete report of dermatologic findings in this population. To fill this knowledge gap, 94 patients with Williams syndrome aged 7-50 years were recruited as part of the skin and vascular elasticity (WS-SAVE) study. They underwent either a clinical dermatologic assessment by trained dermatologists (2010 WSA family meeting) or measurement of biomechanical properties of the skin with the DermaLab™ suction cup (2012 WSA family meeting). Clinical assessment confirmed that soft skin is common in this population (83%), as is premature graying of the hair (80% of those 20 years or older), while wrinkles (92%), and abnormal scarring (33%) were detected in larger than expected proportions. Biomechanical studies detected statistically significant differences in dP (the pressure required to lift the skin), dT (the time required to raise the skin through a prescribed gradient), VE (viscoelasticity), and E (Young's modulus) relative to matched controls. The RT (retraction time) also trended longer but was not significant. The biomechanical differences noted in these patients did not correlate with the presence of vascular defects also attributable to elastin insufficiency (vascular stiffness, hypertension, and arterial stenosis) suggesting the presence of tissue specific modifiers that modulate the impact of elastin insufficiency in each tissue. © 2014 Wiley Periodicals, Inc.

Elastins from patients with Williams-Beuren syndrome and healthy individuals differ on the molecular level

DEFF Research Database (Denmark)

Heinz, Andrea; Huertas, Angela C Mora; Schräder, Christoph U

2016-01-01

Williams-Beuren syndrome (WBS) is a congenital disorder, which involves the heterozygous deletion of the elastin gene and other genes on chromosome 7. Clinical symptoms that are associated with hemizygosity of the essential extracellular matrix protein elastin include premature aging of the skin...... and supravalvular aortic stenosis. However, only little is known about the molecular basis of structural abnormalities in the connective tissue of WBS patients. Therefore, for the first time this study aimed to systematically characterize and compare the structure and amount of elastin present in skin and aortic...... tissue from WBS patients and healthy individuals. Elastin fibers were isolated from tissue biopsies, and it was found that skin of WBS patients contains significantly less elastin compared to skin of healthy individuals. Scanning electron microscopy and mass spectrometric measurements combined...

David Owen WILLIAMS

CERN Multimedia

2006-01-01

Lidy Williams-Oonk and her children Mark & Marietta, being unable to thank everybody individually, would like to express their sincere thanks to friends and colleagues at CERN and abroad for their great help and support, their messages and flowers, as well as their donations to the Ligue Genevoise contre le Cancer, on the death of their beloved husband and father.

MRI Amygdala Volume in Williams Syndrome

Science.gov (United States)

Capitao, Liliana; Sampaio, Adriana; Sampaio, Cassandra; Vasconcelos, Cristiana; Fernandez, Montse; Garayzabal, Elena; Shenton, Martha E.; Goncalves, Oscar F.

2011-01-01

One of the most intriguing characteristics of Williams Syndrome individuals is their hypersociability. The amygdala has been consistently implicated in the etiology of this social profile, particularly given its role in emotional and social behavior. This study examined amygdala volume and symmetry in WS individuals and in age and sex matched…

Investigating the ''social brain'' through Williams syndrome

International Nuclear Information System (INIS)

Nagamine, Masanori; Mimura, Masaru; Reiss, A.L.; Hoeft, F.

2010-01-01

Recent advances in social cognitive neuroscience have led to the concept of the ''social brain''. The social brain includes neural processes specialized for processing social information necessary for the recognition of self and others, and interpersonal relationships. Because of its unique behavioral phenotypic features which includes 'hypersociability', Williams syndrome has gained popularity among social cognitive neuroscientists. Individuals with Williams syndrome share the same genetic risk factor for cognitive-behavioral dysfunction utilizing brain imaging to elucidate endophenotype provides us with an unprecendented opportunity to study gene, brain and behavior relationships especially those related to social cognition. In this review, we provide an overview of neuroimaging studies on social cognition in Williams syndrome and discuss the neural basis of the social brain. (author)

Williams syndrome deletions and duplications: Genetic windows to understanding anxiety, sociality, autism, and schizophrenia.

Science.gov (United States)

Crespi, Bernard J; Procyshyn, Tanya L

2017-08-01

We describe and evaluate an integrative hypothesis for helping to explain the major neurocognitive features of individuals with Williams syndrome region deletions and duplications. First, we demonstrate how the cognitive differences between Williams syndrome individuals, individuals with duplications of this region, and healthy individuals parallel the differences between individuals subject to effects of increased or decreased oxytocin. Second, we synthesize evidence showing that variation in expression of the gene GTF2I (General Transcription Factor II-I) underlies the primary social phenotypes of Williams syndrome and that common genetic variation in GTF2I mediates oxytocin reactivity, and its correlates, in healthy populations. Third, we describe findings relevant to the hypothesis that the GTF2I gene is subject to parent of origin effects whose behavioral expression fits with predictions from the kinship theory of genomic imprinting. Fourth, we describe how Williams syndrome can be considered, in part, as an autistic syndrome of Lorna Wing's 'active-but-odd' autism subtype, in contrast to associations of duplications with both schizophrenia and autism. Copyright © 2017 Elsevier Ltd. All rights reserved.

Fears, Hyperacusis and Musicality in Williams Syndrome

Science.gov (United States)

Blomberg, Stefan; Rosander, Michael; Andersson, Gerhard

2006-01-01

The study investigated the prevalence of fear and hyperacusis and the possible connections between fear, hyperacusis and musicality in a Swedish sample of individuals with Williams syndrome (WS). The study included 38 individuals and a cross-sectional design, with no matched control group. Two persons, who knew the participant well, completed a…

Learning by observation: insights from Williams syndrome.

Science.gov (United States)

Foti, Francesca; Menghini, Deny; Mandolesi, Laura; Federico, Francesca; Vicari, Stefano; Petrosini, Laura

2013-01-01

Observing another person performing a complex action accelerates the observer's acquisition of the same action and limits the time-consuming process of learning by trial and error. Observational learning makes an interesting and potentially important topic in the developmental domain, especially when disorders are considered. The implications of studies aimed at clarifying whether and how this form of learning is spared by pathology are manifold. We focused on a specific population with learning and intellectual disabilities, the individuals with Williams syndrome. The performance of twenty-eight individuals with Williams syndrome was compared with that of mental age- and gender-matched thirty-two typically developing children on tasks of learning of a visuo-motor sequence by observation or by trial and error. Regardless of the learning modality, acquiring the correct sequence involved three main phases: a detection phase, in which participants discovered the correct sequence and learned how to perform the task; an exercise phase, in which they reproduced the sequence until performance was error-free; an automatization phase, in which by repeating the error-free sequence they became accurate and speedy. Participants with Williams syndrome beneficiated of observational training (in which they observed an actor detecting the visuo-motor sequence) in the detection phase, while they performed worse than typically developing children in the exercise and automatization phases. Thus, by exploiting competencies learned by observation, individuals with Williams syndrome detected the visuo-motor sequence, putting into action the appropriate procedural strategies. Conversely, their impaired performances in the exercise phases appeared linked to impaired spatial working memory, while their deficits in automatization phases to deficits in processes increasing efficiency and speed of the response. Overall, observational experience was advantageous for acquiring competencies

Learning by observation: insights from Williams syndrome.

Directory of Open Access Journals (Sweden)

Francesca Foti

Full Text Available Observing another person performing a complex action accelerates the observer's acquisition of the same action and limits the time-consuming process of learning by trial and error. Observational learning makes an interesting and potentially important topic in the developmental domain, especially when disorders are considered. The implications of studies aimed at clarifying whether and how this form of learning is spared by pathology are manifold. We focused on a specific population with learning and intellectual disabilities, the individuals with Williams syndrome. The performance of twenty-eight individuals with Williams syndrome was compared with that of mental age- and gender-matched thirty-two typically developing children on tasks of learning of a visuo-motor sequence by observation or by trial and error. Regardless of the learning modality, acquiring the correct sequence involved three main phases: a detection phase, in which participants discovered the correct sequence and learned how to perform the task; an exercise phase, in which they reproduced the sequence until performance was error-free; an automatization phase, in which by repeating the error-free sequence they became accurate and speedy. Participants with Williams syndrome beneficiated of observational training (in which they observed an actor detecting the visuo-motor sequence in the detection phase, while they performed worse than typically developing children in the exercise and automatization phases. Thus, by exploiting competencies learned by observation, individuals with Williams syndrome detected the visuo-motor sequence, putting into action the appropriate procedural strategies. Conversely, their impaired performances in the exercise phases appeared linked to impaired spatial working memory, while their deficits in automatization phases to deficits in processes increasing efficiency and speed of the response. Overall, observational experience was advantageous for

33 CFR 167.1702 - In Prince William Sound: Prince William Sound Traffic Separation Scheme.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false In Prince William Sound: Prince William Sound Traffic Separation Scheme. 167.1702 Section 167.1702 Navigation and Navigable Waters COAST....1702 In Prince William Sound: Prince William Sound Traffic Separation Scheme. The Prince William Sound...

Interview: William and Barbara Christopher.

Science.gov (United States)

Teaching Exceptional Children, 1986

1986-01-01

Asserting that the biggest mistake educators can make is to set themselves up as experts vis-a-vis parents as amateurs, Barbara and William Christopher emphasize the importance of continued education for the autistic individual and relate their experiences with the educational community as they sought help for their autistic son. (JC)

Colour discrimination and categorisation in Williams syndrome.

Science.gov (United States)

Farran, Emily K; Cranwell, Matthew B; Alvarez, James; Franklin, Anna

2013-10-01

Individuals with Williams syndrome (WS) present with impaired functioning of the dorsal visual stream relative to the ventral visual stream. As such, little attention has been given to ventral stream functions in WS. We investigated colour processing, a predominantly ventral stream function, for the first time in nineteen individuals with Williams syndrome. Colour discrimination was assessed using the Farnsworth-Munsell 100 hue test. Colour categorisation was assessed using a match-to-sample test and a colour naming task. A visual search task was also included as a measure of sensitivity to the size of perceptual colour difference. Results showed that individuals with WS have reduced colour discrimination relative to typically developing participants matched for chronological age; performance was commensurate with a typically developing group matched for non-verbal ability. In contrast, categorisation was typical in WS, although there was some evidence that sensitivity to the size of perceptual colour differences was reduced in this group. Copyright © 2013 Elsevier Ltd. All rights reserved.

Stranger Danger Awareness in Williams Syndrome

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Riby, D. M.; Kirk, H.; Hanley, M.; Riby, L. M.

2014-01-01

Background: The developmental disorder Williams syndrome (WS) is characterised by a distinctive cognitive profile and an intriguing social phenotype. Individuals with the disorder are often highly social engaging with familiar and unfamiliar people and once in an interaction they often show subtle abnormalities of social behaviour. Atypically…

[Williams-Beuren syndrome (Williams syndrome). Case report].

Science.gov (United States)

Miklós, Györgyi; Fekete, György; Haltrich, Irén; Tóth, Miklós; Reismann, Péter

2017-11-01

Williams syndrome is a rare genetic disorder, that occurs equally in all ethnic groups and both sexes. The diagnosis might be missed during childhood in mild cases. However, establishing the diagnosis is important, not only to find the cause of intellectual disability but to look for cardiovascular, endocrine, psychiatry, urology and other conditions, which can occur at any age in the patients' lifetime. This case report presents the story of 47-year-old woman, who was admitted with haematemesis. During her stay on the ward, in the light of the distinctive facial features, mental retardation, and social behaviour patterns, the possibility of Williams syndrome emerged. Later, the diagnosis was confirmed by genetic analysis. This female is the oldest living patient with Williams syndrome in Hungary. Orv Hetil. 2017; 158(47): 1883-1888.

Fading-Figure Tracing in Williams Syndrome

Science.gov (United States)

Nagai, Chiyoko; Inui, Toshio; Iwata, Makoto

2011-01-01

Williams syndrome (WS) is a neurodevelopmental disorder characterized by severe impairment of visuospatial abilities. Figure-drawing abilities, which are thought to reflect visuospatial abilities, have yet to be fully investigated in WS. The purpose of the present study was to clarify whether drawing abilities differ between WS individuals and…

Anxiety Disorders in Williams Syndrome Contrasted with Intellectual Disability and the General Population: A Systematic Review and Meta-Analysis

Science.gov (United States)

Royston, R.; Howlin, P.; Waite, J.; Oliver, C.

2017-01-01

Individuals with specific genetic syndromes associated with intellectual disability (ID), such as Williams syndrome (WS), are at increased risk for developing anxiety disorders. A systematic literature review identified sixteen WS papers that could generate pooled prevalence estimates of anxiety disorders for WS. A meta-analysis compared these…

Perceiving Facial and Vocal Expressions of Emotion in Individuals with Williams Syndrome

Science.gov (United States)

Plesa-Skwerer, Daniela; Faja, Susan; Schofield, Casey; Verbalis, Alyssa; Tager-Flusberg, Helen

2006-01-01

People with Williams syndrome are extremely sociable, empathic, and expressive in communication. Some researchers suggest they may be especially sensitive to perceiving emotional expressions. We administered the Faces and Paralanguage subtests of the Diagnostic Analysis of Nonverbal Accuracy Scale (DANVA2), a standardized measure of emotion…

Description of common musculoskeletal findings in Williams Syndrome and implications for therapies.

Science.gov (United States)

Copes, L E; Pober, B R; Terilli, C A

2016-07-01

Williams syndrome (WS), also referred to as Williams-Beuren syndrome (WBS), is a relatively rare genetic disorder affecting ∼1/10,000 persons. Since the disorder is caused by a micro-deletion of ∼1.5 Mb, it is not surprising that the manifestations of WS are extremely broad, involving most body systems. In this paper, we primarily focus on the musculoskeletal aspects of WS as these findings have not been the subject of a comprehensive review. We review the MSK features commonly seen in individuals with WS, along with related sensory and neurological issues interacting with and compounding underlying MSK abnormalities. We end by providing perspective, particularly from the vantage point of a physical therapist, on therapeutic interventions to address the most common MSK and related features seen in WS. Clin. Anat. 29:578-589, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Binding of visual and spatial short-term memory in Williams syndrome and moderate learning disability.

Science.gov (United States)

Jarrold, Christopher; Phillips, Caroline; Baddeley, Alan D

2007-04-01

A main aim of this study was to test the claim that individuals with Williams syndrome have selectively impaired memory for spatial as opposed to visual information. The performance of 16 individuals with Williams syndrome (six males, 10 females; mean age 18y 7mo [SD 7y 6mo], range 9y 1mo-30y 7mo) on tests of short-term memory for item and location information was compared with that shown by individuals with moderate learning difficulties (12 males, four females; mean age 10y 3mo [SD 1y], range 8y 6mo-11y 7mo) and typically developing children (six males, 10 females; mean age 6y 8mo [SD 7mo], range 5y 10mo-7y 9mo) of an equivalent level of visuospatial ability. A second aim was to determine whether individuals had impaired ability to 'bind' visual spatial information when required to recall 'item in location' information. In contrast to previous findings, there was no evidence that individuals with Williams syndrome were more impaired in the spatial than the visual memory condition. However, individuals with both Williams syndrome and moderate learning difficulties showed impaired memory for item in location information, suggesting that problems of binding may be generally associated with learning disability.

Visually Guided Step Descent in Children with Williams Syndrome

Science.gov (United States)

Cowie, Dorothy; Braddick, Oliver; Atkinson, Janette

2012-01-01

Individuals with Williams syndrome (WS) have impairments in visuospatial tasks and in manual visuomotor control, consistent with parietal and cerebellar abnormalities. Here we examined whether individuals with WS also have difficulties in visually controlling whole-body movements. We investigated visual control of stepping down at a change of…

Ethical Life in Kierkegaard and Williams

OpenAIRE

Stocker, Barry

2018-01-01

A discussion of how the criticisms of ethical theory in Søren Kierkegaard and Bernard Williams both reinforce each other and also provide some challenges to each other. Despite Williams’ brief and dismissive encounter with Kierkegaard around the reading of a ancient tragedy, both oppose any tendency to see the characters in those tragedies as lacking in agency. Both are consistently concerned with how the individual struggles for some ethical agency and how no individual can be free of the in...

Long-term memory for verbal and visual information in Down syndrome and Williams syndrome: performance on the Doors and People test.

Science.gov (United States)

Jarrold, Christopher; Baddeley, Alan D; Phillips, Caroline

2007-02-01

Previous studies have suggested that Williams syndrome and Down syndrome may be associated with specific short-term memory deficits. Individuals with Williams syndrome perform relatively poorly on tests of visuo-spatial short-term memory and individuals with Down syndrome show a relative deficit on verbal short-term memory tasks. However, these patterns of impairments may reflect the impact of generally impaired visuo-spatial processing skills in Williams syndrome, and verbal abilities in Down syndrome. The current study explored this possibility by assessing long-term memory among 15 individuals with Williams syndrome and 20 individuals with Down syndrome using the Doors and People test, a battery which assesses recall and recognition of verbal and visual information. Individuals' performance was standardised for age and level of intellectual ability with reference to that shown by a sample of 110 typically developing children. The results showed that individuals with Down syndrome have no differential deficits in long-term memory for verbal information, implying that verbal short-term memory deficits in this population are relatively selective. Instead both individuals with Down syndrome and with Williams syndrome showed some evidence of relatively poor performance on tests of long-term memory for visual information. It is therefore possible that visuo-spatial short-term memory deficits that have previously been demonstrated in Williams syndrome may be secondary to more general problems in visuo-spatial processing in this population.

Binding of Visual and Spatial Short-Term Memory in Williams Syndrome and Moderate Learning Disability

Science.gov (United States)

Jarrold, Christopher; Phillips, Caroline; Baddeley, Alan D

2007-01-01

A main aim of this study was to test the claim that individuals with Williams syndrome have selectively impaired memory for spatial as opposed to visual information. The performance of 16 individuals with Williams syndrome (six males, 10 females; mean age 18y 7mo [SD 7y 6mo], range 9y 1mo-30y 7mo) on tests of short-term memory for item and…

Dual-Task Processing as a Measure of Executive Function: A Comparison between Adults with Williams and Down Syndromes

Science.gov (United States)

Kittler, Phyllis M.; Krinsky-McHale, Sharon J.; Devenny, Darlynne A.

2008-01-01

Behavioral phenotypes of individuals with Williams syndrome and individuals with Down syndrome have been contrasted in relation to short-term memory. People with Down syndrome are stronger visuospatially and those with Williams syndrome are stronger verbally. We examined short-term memory, then explored whether dual-task processing further…

MacWilliams Identity for M-Spotty Weight Enumerator

Science.gov (United States)

Suzuki, Kazuyoshi; Fujiwara, Eiji

M-spotty byte error control codes are very effective for correcting/detecting errors in semiconductor memory systems that employ recent high-density RAM chips with wide I/O data (e.g., 8, 16, or 32bits). In this case, the width of the I/O data is one byte. A spotty byte error is defined as random t-bit errors within a byte of length b bits, where 1 le t ≤ b. Then, an error is called an m-spotty byte error if at least one spotty byte error is present in a byte. M-spotty byte error control codes are characterized by the m-spotty distance, which includes the Hamming distance as a special case for t =1 or t = b. The MacWilliams identity provides the relationship between the weight distribution of a code and that of its dual code. The present paper presents the MacWilliams identity for the m-spotty weight enumerator of m-spotty byte error control codes. In addition, the present paper clarifies that the indicated identity includes the MacWilliams identity for the Hamming weight enumerator as a special case.

The use of emotions in narratives in Williams syndrome.

Science.gov (United States)

Van Herwegen, Jo; Aznar, Ana; Tenenbaum, Harriet

2014-01-01

Although individuals with Williams syndrome are very sociable, they tend to have limited contact and friendships with peers. In typically developing children the use of positive emotions (e.g., happy) has been argued to be related to peer relationships and popularity. The current study investigated the use and development of emotion words in Williams syndrome using cross-sectional developmental trajectories and examined children's use of different types of emotion words. Nineteen children with Williams syndrome (WS) and 20 typically developing (TD) children matched for chronological age told a story from a wordless picture book. Participants with WS produced a similar number of emotion words compared to the control group and the use of emotion words did not change when plotted against chronological age or vocabulary abilities in either group. However, participants with WS produced more emotion words about sadness. Links between emotion production and friendships as well as future studies are discussed. After reading this article, readers will be able to: explain the development of positive and negative emotions in Williams syndrome and recognize that emotion production is atypical in this population. Copyright © 2014 Elsevier Inc. All rights reserved.

Examining Reports of Mental Health in Adults with Williams Syndrome

Science.gov (United States)

Stinton, Chris; Tomlinson, Katie; Estes, Zachary

2012-01-01

Prior research suggests that individuals with Williams syndrome (WS) have a disposition towards anxiety. Information regarding this is typically derived from parents and carers. The perspectives of the individuals with WS are rarely included in research of this nature. We examined the mental health of 19 adults with WS using explicit (psychiatric…

N-acetylcysteine for neuropsychiatric symptoms in a woman with Williams syndrome.

Science.gov (United States)

Pineiro, Mildred Lopez; Roberts, Antoinette M; Waxler, Jessica L; Mullett, Jennifer E; Pober, Barbara R; McDougle, Christopher J

2014-11-01

Williams syndrome is a relatively rare genetic disorder caused by the hemizygous microdeletion of a region in chromosome 7q11.23. Individuals with Williams syndrome typically present with a highly social, overfriendly, and empathic personality. Comorbid medical and neuropsychiatric disorders are common. Reports of effective pharmacological treatment of associated neuropsychiatric disorders are limited. The authors describe the successful treatment of interfering anger, aggression, and hair-pulling with N-acetylcysteine in a 19-year-old woman with Williams syndrome. The neuropsychiatric symptoms emerged 1 week following an upper gastrointestinal endoscopy, for which fentanyl, midazolam, and propofol were used as anesthetics. The patient's treatment course and hypothesized mechanisms underlying the clinical presentation and symptom resolution are described. © The Author(s) 2014.

William Shakespeare’s“Hamlet”and Oedipus Complex

Institute of Scientific and Technical Information of China (English)

迪丽努尔

2014-01-01

“Hamlet”is one of the great dramas of William Shakespeare. This paper by describing the relationship between Hamlet and his mother, the Ghost and his uncle, tries to approve that Oedipus complex is the main reason of Hamlet’s kil ing his uncle.

Adaptive Functioning in Williams Syndrome: A Systematic Review

Science.gov (United States)

Brawn, Gabrielle; Porter, Melanie

2018-01-01

Literature on the level of adaptive functioning and relative strengths and weaknesses in functioning of individuals with Williams syndrome (WS) was reviewed. The electronic databases PsycINFO, PubMed, Expanded Academic, Web of Science, Scopus and ProQuest were searched electronically for relevant articles and dissertations using the search terms…

Orientation Perception in Williams Syndrome: Discrimination and Integration

Science.gov (United States)

Palomares, Melanie; Landau, Barbara; Egeth, Howard

2009-01-01

Williams Syndrome (WS) is a rare neurodevelopmental disorder, which stems from a genetic deletion on chromosome 7 and causes a profound weakness in visuospatial cognition. Our current study explores how orientation perception may contribute to the visuospatial deficits in WS. In Experiment 1, we found that WS individuals and normal 3-4 year olds…

Gender Attribution and Gender Agreement in French Williams Syndrome

Science.gov (United States)

Boloh, Yves; Ibernon, Laure; Royer, Stephanie; Escudier, Frederique; Danillon, Aurelia

2009-01-01

Previous studies on grammatical gender in French individuals with Williams syndrome (WS) have led to conflicting findings and interpretations regarding keys abilities--gender attribution and gender agreement. New production data from a larger SW sample (N = 24) showed that gender attribution scores in SW participants exactly mirrored those of…

Contrast-Marking Prosodic Emphasis in Williams Syndrome: Results of Detailed Phonetic Analysis

Science.gov (United States)

Ito, Kiwako; Martens, Marilee A.

2017-01-01

Background: Past reports on the speech production of individuals with Williams syndrome (WS) suggest that their prosody is anomalous and may lead to challenges in spoken communication. While existing prosodic assessments confirm that individuals with WS fail to use prosodic emphasis to express contrast, those reports typically lack detailed…

Evaluation of the influence of clinical symptoms on the quality of life in individuals with Williams-Beuren Syndrome

Directory of Open Access Journals (Sweden)

Andrea Parolin Jackowski

2017-03-01

Full Text Available Introduction: Williams-Beuren Syndrome (WBS results from a deletion in the chromosome 7q11.23. Psychiatric symptoms, such as Generalized Anxiety Disorder (GAD, Attention Deficit Hyperactivity Disorder (ADHD, and others, have been described in this syndrome. Objective: The main purpose of this study was to identify related clinical symptoms in individuals with WBS and the impact of this disorder in their quality of life. Method: The study sample included 42 individuals aged 11 to 16 years divided into two groups: a study group composed of 20 individuals with WBS and a control group comprising 22 individuals with typical development. The following instruments were used in this evaluation: Brazilian Criterion of Economic Classification (CCEB, to estimate the purchasing power of urban households; Strengths & Difficulties Questionnaire (SDQ, to provide a useful measure of psychopathology applied to children and youth aged 4-17 years; and Quality of Life Evaluation Scale (AUQEI, to assess the feeling of well-being according to the opinion of the child and adolescent. Results: The results suggest that adolescents with WBS have a good quality of life despite presenting greater possibilities of psychiatric symptoms. These results are valid considering that quality of life is measured from the opinion of the assessed individual. Conclusion: We hypothesized that adolescents with WBS can present a distortion of reality regarding the positive/optimistic factor, possibly based on some characteristics of the syndrome such as being always cheerful and smiling, which are factors associated with intellectual disability.

Beyond Behaviour: Is Social Anxiety Low in Williams Syndrome?

Science.gov (United States)

Dodd, Helen F.; Schniering, Carolyn A.; Porter, Melanie A.

2009-01-01

Individuals with Williams syndrome (WS) exhibit striking social behaviour that may be indicative of abnormally low social anxiety. The present research aimed to determine whether social anxiety is unusually low in WS and to replicate previous findings of increased generalised anxiety in WS using both parent and self report. Fifteen individuals…

Language and Literacy Development of Children with Williams Syndrome

Science.gov (United States)

Mervis, Carolyn B.

2009-01-01

Williams syndrome is a rare neurodevelopmental disorder caused by deletion of approximately 25 genes on chromosome 7q11.23. Children with the syndrome evidence large individual differences in both broad language and reading abilities. Nevertheless, as a group, children with this syndrome show a consistent pattern characterized by relative…

Musicality Correlates with Sociability and Emotionality in Williams Syndrome

Science.gov (United States)

Ng, Rowena; Lai, Philip; Levitin, Daniel J.; Bellugi, Ursula

2013-01-01

Williams syndrome (WS) is a neurogenetic developmental disorder characterized by peaks and valleys of cognitive abilities. One peak that has been understudied is the affinity that many individuals with WS have with music. It remains unknown whether their high levels of musical interest, skill, and expressivity are related to their sociable…

Brief Report: Repetitive Behaviour Profiles in Williams Syndrome: Cross Syndrome Comparisons with Prader-Willi and Down Syndromes

Science.gov (United States)

Royston, R.; Oliver, C.; Moss, J.; Adams, D.; Berg, K.; Burbidge, C.; Howlin, P.; Nelson, L.; Stinton, C.; Waite, J.

2018-01-01

This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were…

The Development of Metaphorical Language Comprehension in Typical Development and in Williams Syndrome

Science.gov (United States)

Thomas, Michael S. C.; Van Duuren, Mike; Purser, Harry R. M.; Mareschal, Denis; Ansari, Daniel; Karmiloff-Smith, Annette

2010-01-01

The domain of figurative language comprehension was used to probe the developmental relation between language and cognition in typically developing individuals and individuals with Williams syndrome. Extending the work of Vosniadou and Ortony, the emergence of nonliteral similarity and category knowledge was investigated in 117 typically…

William Butler Yeats’s ‘The Symbolic System’ of William Blake

Directory of Open Access Journals (Sweden)

Arianna Antonielli

2008-03-01

Full Text Available The theosophical systems formulated by great poets, such as William Blake and William Butler Yeats, represent a personal idiosyncratic actualization of an ancient repertoire of magical symbols and occult visions. This study wants to focus the attention on the philosophical, mythical, and esoteric syncretism that W. B. Yeats drew from William Blake’s symbolical system. A fundamental step of Yeats’s deep investigation into the Blakean ‘vision’ was given by his monumental work, written together with Edwin John Ellis, on Blake’s poetic and pictorial production, completed in 1893 with a three-volume edition entitled The Works of William Blake, Poetic, Symbolic, and Critical. This work, published in London by Bernard Quaritch, deeply influenced Yeats’s symbolical and imaginary system, determining its subsequent development up to its codification in the volume of A Vision. With WWB, Yeats was able to systematize for the first time his own thought, giving unity to his Weltanschauung and his poetry. Following this hypothesis, I concentrated on Yeats’s and Ellis’s numerous analyses dedicated to Blake’s mythological and symbolical corpus and, in particular, I examined the last chapter of the first volume of the Quaritch edition. This chapter, entitled “The Symbolic System”, constitutes an unquestionable link between Yeats the reader and scholar of Blake, and Yeats the poet and follower of Blake.

Caregiver Survey of Pharmacotherapy to Treat Attention Deficit/Hyperactivity Disorder in Individuals with Williams Syndrome

Science.gov (United States)

Martens, Marilee A.; Seyfer, Daisha L.; Andridge, Rebecca R.; Foster, Jessica E. A.; McClure, Kelsey E.; Coury, Daniel L.

2013-01-01

Williams syndrome (WS) is a genetic condition characterized by a unique neurocognitive and behavioral profile, including increased incidence of attention deficit/hyperactivity disorder (ADHD). The purpose of the present study was to examine the perceived helpfulness and side effects of medications used to treat ADHD (methylphenidate class,…

Clinical utility of an array comparative genomic hybridization analysis for Williams syndrome.

Science.gov (United States)

Yagihashi, Tatsuhiko; Torii, Chiharu; Takahashi, Reiko; Omori, Mikimasa; Kosaki, Rika; Yoshihashi, Hiroshi; Ihara, Masahiro; Minagawa-Kawai, Yasuyo; Yamamoto, Junichi; Takahashi, Takao; Kosaki, Kenjiro

2014-11-01

To reveal the relation between intellectual disability and the deleted intervals in Williams syndrome, we performed an array comparative genomic hybridization analysis and standardized developmental testing for 11 patients diagnosed as having Williams syndrome based on fluorescent in situ hybridization testing. One patient had a large 4.2-Mb deletion spanning distally beyond the common 1.5-Mb intervals observed in 10/11 patients. We formulated a linear equation describing the developmental age of the 10 patients with the common deletion; the developmental age of the patient with the 4.2-Mb deletion was significantly below the expectation (developmental age = 0.51 × chronological age). The large deletion may account for the severe intellectual disability; therefore, the use of array comparative genomic hybridization may provide practical information regarding individuals with Williams syndrome. © 2014 Japanese Teratology Society.

Gaze Aversion during Social Style Interactions in Autism Spectrum Disorder and Williams Syndrome

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Doherty-Sneddon, Gwyneth; Whittle, Lisa; Riby, Deborah M.

2013-01-01

During face-to-face interactions typically developing individuals use gaze aversion (GA), away from their questioner, when thinking. GA is also used when individuals with autism (ASD) and Williams syndrome (WS) are thinking during question-answer interactions. We investigated GA strategies during face-to-face social style interactions with…

Comprehension of Metaphor and Metonymy in Children with Williams Syndrome

Science.gov (United States)

Annaz, Dagmara; Van Herwegen, Jo; Thomas, Michael; Fishman, Roza; Karmiloff-Smith, Annette; Rundblad, Gabriella

2009-01-01

Background: Figurative language, such as metaphor and metonymy, is very common in daily language use. Its underlying cognitive processes are sometimes viewed as lying at the interface of language and thought. Williams syndrome, which is a rare genetic developmental disorder, provides an opportunity to study this interface because individuals with…

Attention Bias to Emotional Faces Varies by IQ and Anxiety in Williams Syndrome

Science.gov (United States)

McGrath, Lauren M.; Oates, Joyce M.; Dai, Yael G.; Dodd, Helen F.; Waxler, Jessica; Clements, Caitlin C.; Weill, Sydney; Hoffnagle, Alison; Anderson, Erin; MacRae, Rebecca; Mullett, Jennifer; McDougle, Christopher J.; Pober, Barbara R.; Smoller, Jordan W.

2016-01-01

Individuals with Williams syndrome (WS) often experience significant anxiety. A promising approach to anxiety intervention has emerged from cognitive studies of attention bias to threat. To investigate the utility of this intervention in WS, this study examined attention bias to happy and angry faces in individuals with WS (N = 46). Results showed…

Human versus Non-Human Face Processing: Evidence from Williams Syndrome

Science.gov (United States)

Santos, Andreia; Rosset, Delphine; Deruelle, Christine

2009-01-01

Increased motivation towards social stimuli in Williams syndrome (WS) led us to hypothesize that a face's human status would have greater impact than face's orientation on WS' face processing abilities. Twenty-nine individuals with WS were asked to categorize facial emotion expressions in real, human cartoon and non-human cartoon faces presented…

Personal Space Regulation in Williams Syndrome: The Effect of Familiarity

Science.gov (United States)

Lough, Emma; Flynn, Emma; Riby, Deborah M.

2016-01-01

Personal space refers to a protective barrier that we strive to maintain around our body. We examined personal space regulation in young people with Williams syndrome (WS) and their typically developing, chronological age-matched peers using a parent report questionnaire and a stop-distance paradigm. Individuals with WS were reported by their…

The Interplay between Executive Control and Motor Functioning in Williams Syndrome

Science.gov (United States)

Hocking, Darren R.; Thomas, Daniel; Menant, Jasmine C.; Porter, Melanie A.; Smith, Stuart; Lord, Stephen R.; Cornish, Kim M.

2013-01-01

Previous studies suggest that individuals with Williams syndrome (WS), a rare genetically based neurodevelopmental disorder, show specific weaknesses in visual attention and response inhibition within the visuospatial domain. Here we examine the extent to which impairments in attentional control extend to the visuomotor domain using a…

How Executive Functions Are Related to Intelligence in Williams Syndrome

Science.gov (United States)

Osorio, Ana; Cruz, Raquel; Sampaio, Adriana; Garayzabal, Elena; Martinez-Regueiro, Rocio; Goncalves, Oscar F.; Carracedo, Angel; Fernandez-Prieto, Montse

2012-01-01

Williams syndrome is characterized by impairments in executive functions (EFs). However, it remains unknown how distinct types of EFs relate to intelligence in this syndrome. The present study analyzed performance on working memory, inhibiting and shifting, and its links to IQ in a sample of 17 individuals with WS, and compared them with a group…

Arterial Hypertension in a Child with Williams-Beuren Syndrome (7q11.23 Chromosomal Deletion

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Cristina de Sylos

2002-08-01

Full Text Available We report the case of a 7-year-old male child diagnosed with Williams-Beuren syndrome and arterial hypertension refractory to clinical treatment. The diagnosis was confirmed by genetic study. Narrowing of the descending aorta and stenosis of the renal arteries were also diagnosed. Systemic vascular alterations caused by deletion of the elastin gene may occur early in individuals with Williams-Beuren syndrome, leading to the clinical manifestation of systemic arterial hypertension refractory to drug treatment.

33 CFR 167.1700 - In Prince William Sound: General.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false In Prince William Sound: General... Schemes and Precautionary Areas Pacific West Coast § 167.1700 In Prince William Sound: General. The Prince William Sound Traffic Separation Scheme consists of four parts: Prince William Sound Traffic Separation...

Cognitive-behavioral phenotypes of Williams syndrome are associated with genetic variation in the GTF2I gene, in a healthy population.

Science.gov (United States)

Crespi, Bernard J; Hurd, Peter L

2014-11-28

Individuals with Williams syndrome, a neurogenetic condition caused by deletion of a set of genes at chromosomal location 7q11.23, exhibit a remarkable suite of traits including hypersociality with high, nonselective friendliness and low social anxiety, expressive language relatively well-developed but under-developed social-communication skills overall, and reduced visual-spatial abilities. Deletions and duplications of the Williams-syndrome region have also been associated with autism, and with schizophrenia, two disorders centrally involving social cognition. Several lines of evidence have linked the gene GTF2I (General Transcription Factor IIi) with the social phenotypes of Williams syndrome, but a role for this gene in sociality within healthy populations has yet to be investigated. We genotyped a large set of healthy individuals for two single-nucleotide polymorphisms in the GTF2I gene that have recently been significantly associated with autism, and thus apparently exhibit functional effects on autism-related social phenotypes. GTF2I genotypes for these SNPs showed highly significant association with low social anxiety combined with reduced social-communication abilities, which represents a metric of the Williams-syndrome cognitive profile as described from previous studies. These findings implicate the GTF2I gene in the neurogenetic basis of social communication and social anxiety, both in Williams syndrome and among individuals in healthy populations.

William Rowan Hamilton: Mathematical genius

International Nuclear Information System (INIS)

Wilkins, D.R.

2006-01-01

This year Ireland celebrates the bicentenary of the mathematician William Rowan Hamilton, best remembered for quaternions and for his pioneering work on optics and dynamics. Two centuries after his birth, the extent to which terms such as Hamiltonian and Hamiltonian system have entered the everyday language of mathematicians and physicists testifies to the continuing impact of the scientific work of William Rowan Hamilton. (U.K.)

Individuality evaluation for paper based artifact-metrics using transmitted light image

Science.gov (United States)

Yamakoshi, Manabu; Tanaka, Junichi; Furuie, Makoto; Hirabayashi, Masashi; Matsumoto, Tsutomu

2008-02-01

Artifact-metrics is an automated method of authenticating artifacts based on a measurable intrinsic characteristic. Intrinsic characters, such as microscopic random-patterns made during the manufacturing process, are very difficult to copy. A transmitted light image of the distribution can be used for artifact-metrics, since the fiber distribution of paper is random. Little is known about the individuality of the transmitted light image although it is an important requirement for intrinsic characteristic artifact-metrics. Measuring individuality requires that the intrinsic characteristic of each artifact significantly differs, so having sufficient individuality can make an artifact-metric system highly resistant to brute force attack. Here we investigate the influence of paper category, matching size of sample, and image-resolution on the individuality of a transmitted light image of paper through a matching test using those images. More concretely, we evaluate FMR/FNMR curves by calculating similarity scores with matches using correlation coefficients between pairs of scanner input images, and the individuality of paper by way of estimated EER with probabilistic measure through a matching method based on line segments, which can localize the influence of rotation gaps of a sample in the case of large matching size. As a result, we found that the transmitted light image of paper has a sufficient individuality.

Absolutism and Natural Law Argument: William O. Douglas on Freedom of Expression.

Science.gov (United States)

Rodgers, Raymond S.

Noting that United States Supreme Court Justice William O. Douglas has often been characterized as an "absolutist" in terms of First Amendment policy, this paper argues that, in fact, Douglas's policy positions provided for less than absolute freedom to communicate. The paper then reveals, through an anlaysis of 18 of Douglas's opinions,…

Development of Novel Metaphor and Metonymy Comprehension in Typically Developing Children and Williams Syndrome

Science.gov (United States)

Van Herwegen, Jo; Dimitriou, Dagmara; Rundblad, Gabriella

2013-01-01

This study investigated the development of novel metaphor and metonymy comprehension in both typically developing (TD) children and individuals with Williams syndrome (WS). Thirty-one TD children between the ages of 3;09 and 17;01 and thirty-four individuals with WS between the ages of 7;01 and 44 years old were administered a newly developed task…

Toddlers with Williams Syndrome Process Upright but Not Inverted Faces Holistically

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Cashon, Cara H.; Ha, Oh-Ryeong; DeNicola, Christopher A.; Mervis, Carolyn B.

2013-01-01

Holistic processing of upright, but not inverted, faces is a marker of perceptual expertise for faces. This pattern is shown by typically developing individuals beginning at age 7 months. Williams syndrome (WS) is a rare neurogenetic developmental disorder characterized by extreme interest in faces from a very young age. Research on the effects of…

A Comparison between Linguistic Skills and Socio-Communicative Abilities in Williams Syndrome

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Alfieri, P.; Menghini, D.; Marotta, L.; De Peppo, L.; Ravà, L.; Salvaguardia, F.; Varuzza, C.; Vicari, S.

2017-01-01

Background: Individuals with Williams syndrome (WS) show a disharmonic linguistic profile with a clear pattern of strengths and weaknesses. Despite their sociable nature, atypical socio-communicative abilities and deficits in communication and relationship with others have been found. Aim: The aim of the present study was to investigate whether…

Evaluation of a Stranger Safety Training Programme for Adults with Williams Syndrome

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Fisher, M. H.

2014-01-01

Background: Individuals with Williams syndrome (WS) are reported to display increased sociability towards strangers, leading to increased social vulnerability. No research has examined real life interactions of adults with WS towards strangers and no interventions have been implemented to teach stranger safety skills to this population. Method:…

William Rowan Hamilton: Mathematical genius

Energy Technology Data Exchange (ETDEWEB)

Wilkins, D.R. [School of Mathematics, Trinity College, Dublin (Ireland)]. E-mail: dwilkins@maths.tcd.ie

2005-08-01

This year Ireland celebrates the bicentenary of the mathematician William Rowan Hamilton, best remembered for 'quaternions' and for his pioneering work on optics and dynamics. Two centuries after his birth, the extent to which terms such as 'Hamiltonian' and 'Hamiltonian system' have entered the everyday language of mathematicians and physicists testifies to the continuing impact of the scientific work of William Rowan Hamilton. (U.K.)

33 CFR 110.233 - Prince William Sound, Alaska.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 1 2010-07-01 2010-07-01 false Prince William Sound, Alaska. 110... ANCHORAGES ANCHORAGE REGULATIONS Anchorage Grounds § 110.233 Prince William Sound, Alaska. (a) The anchorage grounds. In Prince William Sound, Alaska, beginning at a point at latitude 60°40′00″ N., longitude 146°40...

Williams Syndrome with a “Twist”

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Despoina Maritsi

2010-01-01

Full Text Available Williams syndrome is a rare genetic condition with multisystemic involvement, caused by a microscopic deletion in the chromosome band 7q11.23. We describe the first case of a toddler with Williams syndrome who developed Benign Paroxysmal Torticollis (BPT, a benign dystonic disorder of unknown aetiology.

76 FR 1130 - Prince William Sound Resource Advisory Committee

Science.gov (United States)

2011-01-07

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... conducted: The Prince William Sound Resource Advisory Committee (RAC) will be discussing and voting on...

77 FR 45331 - Prince William Sound Resource Advisory Committee

Science.gov (United States)

2012-07-31

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... Prince William Sound Resource Advisory Committee (RAC) will be discussing and voting on proposals that...

Williams Syndrome Hypersociability: A Neuropsychological Study of the Amygdala and Prefrontal Cortex Hypotheses

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Capitao, Liliana; Sampaio, Adriana; Fernandez, Montse; Sousa, Nuno; Pinheiro, Ana; Goncalves, Oscar F.

2011-01-01

Individuals with Williams syndrome display indiscriminate approach towards strangers. Neuroimaging studies conducted so far have linked this social profile to structural and/or functional abnormalities in WS amygdala and prefrontal cortex. In this study, the neuropsychological hypotheses of amygdala and prefrontal cortex involvement in WS…

Ending Caleb Williams: on storytelling influencing William Godwin’s politics

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Peterson Roberto da Silva

2017-12-01

Full Text Available William Godwin escreveu o romance gótico Things as They Are; or, the Adventures of Caleb Williams (“As Coisas como Elas São; ou, as Aventuras de Caleb Williams”, em tradução livre em 1794 para disseminar suas ideias filosóficas e políticas. Inicio o artigo revisando a literatura para demonstrar como sua teoria influenciou tanto sua decisão de escrever ficções quanto o tipo de ficção que ele escreveu, mostrando como o autor usou e inovou convenções do gênero gótico para transmitir suas ideias para um público mais amplo. Então comparo duas perspectivas divergentes na literatura quanto à maneira como suas narrativas influenciaram suas ideias políticas e filosóficas, em particular a partir de comentários acerca do significado e do impacto de mudanças de última hora ao desfecho de Caleb Williams. Argumento que essa relação ambígua entre narrativa e política na obra de Godwin é relevante para reflexões políticas contemporâneas sobre a relação entre representação e ação política, ou como narrativas sobre a condição humana e realidades políticas podem afetas as ideias, atitudes e relações sociais de seus narradores.

Perfil comunicativo de indivíduos com a síndrome de Williams-Beuren Communicative profile of individuals with Williams-Beuren syndrome

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Natalia Freitas Rossi

2007-03-01

Full Text Available OBJETIVO: Descrever o perfil comunicativo de indivíduos com a síndrome de Williams-Beuren. MÉTODOS: A casuística foi composta por 12 indivíduos com a síndrome com idade cronológica entre 6;6 a 23;6 (Grupo 1 que foram comparados a outros 12 sem a síndrome e com idade mental semelhante e sem dificuldades de linguagem/aprendizagem (Grupo 2. Os indivíduos foram avaliados em situação de conversação para classificação dos comportamentos verbais e não-verbais, segundo critérios pragmáticos, levantamento do número de turnos por minuto, enunciados por turno, Extensão Média de Enunciados, levantamento quanto à freqüência e tipologia de disfluências da fala e classificação quanto ao tipo de pausas plenas do discurso. RESULTADOS: O perfil comunicativo do Grupo 1 mostrou facilidade para interagirem em situação de comunicação com a presença de limitações lingüísticas estruturais e funcionais variáveis, quando comparados aos indivíduos do Grupo 2. Os indivíduos do Grupo 1 freqüentemente utilizaram estratégias comunicativas, na tentativa de preencherem o espaço comunicativo, como o uso de clichês, efeitos sonoros, recursos entonacionais e as pausas plenas que mostraram ser favoráveis do ponto de vista sócio-comunicativo, enquanto que os comportamentos verbais ecolálicos e perseverativos prejudicam o desempenho comunicativo desses indivíduos. CONCLUSÃO: O desempenho comunicativo mais prejudicado do Grupo 1 permitiu especular que comprometimentos lingüísticos nesta condição podem estar presentes, independente da diferença entre idade cronológica e mental. Estudos mais abrangentes poderão responder ao questionamento da dissociação de habilidades cognitivas e lingüísticas nesta síndrome e também no que diz respeito à complexa esta correlação em meio aos distúrbios da comunicação humana.PURPOSE: To describe the communicative profile of individuals with Williams-Beuren syndrome. METHODS: The sample

76 FR 18715 - Prince William Sound Resource Advisory Committee

Science.gov (United States)

2011-04-05

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... meeting is open to the public. The following business will be conducted: The Prince William Sound Resource...

Longitudinal Trajectories of Intellectual and Adaptive Functioning in Adolescents and Adults with Williams Syndrome

Science.gov (United States)

Fisher, M. H.; Lense, M. D.; Dykens, E. M.

2016-01-01

Background: Williams syndrome (WS) is associated with a distinct cognitive-behavioural phenotype including mild to moderate intellectual disability, visual-spatial deficits, hypersociability, inattention and anxiety. Researchers typically characterise samples of individuals with WS by their intellectual functioning and adaptive behaviour. Because…

Mental Health Concerns in Williams Syndrome: Intervention Considerations and Illustrations from Case Examples

Science.gov (United States)

Phillips, Kristin D.; Klein-Tasman, Bonita P.

2009-01-01

The refinement of the Williams syndrome phenotype has frequently included the study of behavioral and temperamental features common to individuals with this disorder. Within this line of research, the importance of evaluating incidence of psychopathology has been increasingly recognized, with studies consistently identifying an increased risk for…

Sleep Patterns and Daytime Sleepiness in Adolescents and Young Adults with Williams Syndrome

Science.gov (United States)

Goldman, S. E.; Malow, B. A.; Newman, K. D.; Roof, E.; Dykens, E. M.

2009-01-01

Background: Sleep disorders are common in individuals with neurodevelopmental disorders and may adversely affect daytime functioning. Children with Williams syndrome have been reported to have disturbed sleep; however, no studies have been performed to determine if these problems continue into adolescence and adulthood. Methods: This study…

Williams propylene upgrading

Energy Technology Data Exchange (ETDEWEB)

Chappell, D. [Williams Energy Canada Inc., Edmonton, AB (Canada)

2004-07-01

Edmonton-based Williams Energy Canada Inc. extracts petrochemicals from oil sands and operates a straddle plant business and an olefins business. This presentation provided an update of both businesses and reviewed the advantage of polypropylene production in Alberta, with reference to premium markets and to comparative rail costs to Chicago via Texas, and rail costs to Chicago from Alberta. Williams' straddle plant business includes the Cochrane Straddle Plant, the Empress 2 Straddle Plant, and the Empress 5 Straddle Plant. The Fort McMurray Extraction Plant was also described along with the Redwater Olefins Fractionator and its potential for salt cavern storage and distribution. It was noted that Alberta is well positioned for polypropylene production because it already has a secure supply and an excellent distribution network. tabs., figs.

Williams propylene upgrading

International Nuclear Information System (INIS)

Chappell, D.

2004-01-01

Edmonton-based Williams Energy Canada Inc. extracts petrochemicals from oil sands and operates a straddle plant business and an olefins business. This presentation provided an update of both businesses and reviewed the advantage of polypropylene production in Alberta, with reference to premium markets and to comparative rail costs to Chicago via Texas, and rail costs to Chicago from Alberta. Williams' straddle plant business includes the Cochrane Straddle Plant, the Empress 2 Straddle Plant, and the Empress 5 Straddle Plant. The Fort McMurray Extraction Plant was also described along with the Redwater Olefins Fractionator and its potential for salt cavern storage and distribution. It was noted that Alberta is well positioned for polypropylene production because it already has a secure supply and an excellent distribution network. tabs., figs

Williams-Beuren's Syndrome: A Case Report.

Science.gov (United States)

Zamani, Hassan; Babazadeh, Kazem; Fattahi, Saeid; Mokhtari-Esbuie, Farzad

2012-01-01

Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD), skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6) in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH) was performed and the result was: 46.XX, ish del (7q11.2) (ELN X1) (7q22 X2) ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

Williams-Beuren's Syndrome: A Case Report

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Hassan Zamani

2012-01-01

Full Text Available Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD, skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6 in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH was performed and the result was: 46.XX, ish del (7q11.2 (ELN X1 (7q22 X2 ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

A Diagnosis to Consider in an Adult Patient with Facial Features and Intellectual Disability: Williams Syndrome.

Science.gov (United States)

Doğan, Özlem Akgün; Şimşek Kiper, Pelin Özlem; Utine, Gülen Eda; Alikaşifoğlu, Mehmet; Boduroğlu, Koray

2017-03-01

Williams syndrome (OMIM #194050) is a rare, well-recognized, multisystemic genetic condition affecting approximately 1/7,500 individuals. There are no marked regional differences in the incidence of Williams syndrome. The syndrome is caused by a hemizygous deletion of approximately 28 genes, including ELN on chromosome 7q11.2. Prenatal-onset growth retardation, distinct facial appearance, cardiovascular abnormalities, and unique hypersocial behavior are among the most common clinical features. Here, we report the case of a patient referred to us with distinct facial features and intellectual disability, who was diagnosed with Williams syndrome at the age of 37 years. Our aim is to increase awareness regarding the diagnostic features and complications of this recognizable syndrome among adult health care providers. Williams syndrome is usually diagnosed during infancy or childhood, but in the absence of classical findings, such as cardiovascular anomalies, hypercalcemia, and cognitive impairment, the diagnosis could be delayed. Due to the multisystemic and progressive nature of the syndrome, accurate diagnosis is critical for appropriate care and screening for the associated morbidities that may affect the patient's health and well-being.

Relations between social-perceptual ability in multi- and unisensory contexts, autonomic reactivity, and social functioning in individuals with Williams syndrome.

Science.gov (United States)

Järvinen, Anna; Ng, Rowena; Crivelli, Davide; Arnold, Andrew J; Woo-VonHoogenstyn, Nicholas; Bellugi, Ursula

2015-07-01

Compromised social-perceptual ability has been proposed to contribute to social dysfunction in neurodevelopmental disorders. While such impairments have been identified in Williams syndrome (WS), little is known about emotion processing in auditory and multisensory contexts. Employing a multidimensional approach, individuals with WS and typical development (TD) were tested for emotion identification across fearful, happy, and angry multisensory and unisensory face and voice stimuli. Autonomic responses were monitored in response to unimodal emotion. The WS group was administered an inventory of social functioning. Behaviorally, individuals with WS relative to TD demonstrated impaired processing of unimodal vocalizations and emotionally incongruent audiovisual compounds, reflecting a generalized deficit in social-auditory processing in WS. The TD group outperformed their counterparts with WS in identifying negative (fearful and angry) emotion, with similar between-group performance with happy stimuli. Mirroring this pattern, electrodermal activity (EDA) responses to the emotional content of the stimuli indicated that whereas those with WS showed the highest arousal to happy, and lowest arousal to fearful stimuli, the TD participants demonstrated the contrasting pattern. In WS, more normal social functioning was related to higher autonomic arousal to facial expressions. Implications for underlying neural architecture and emotional functions are discussed. Copyright © 2015 Elsevier Ltd. All rights reserved.

Cognitive, Linguistic and Adaptive Functioning in Williams Syndrome: Trajectories from Early to Middle Adulthood

Science.gov (United States)

Howlin, Patricia; Elison, Sarah; Udwin, Orlee; Stinton, Christopher

2010-01-01

Background: Little is known about trajectories of cognitive functioning as individuals with Williams syndrome (WS) move though adulthood. Method: The present study investigated cognitive, linguistic and adaptive functioning in adults with WS aged 19-55 years, using both cross-sectional and longitudinal approaches. Results: Data from the…

William Wilde: Historian.

Science.gov (United States)

Geary, L

2016-05-01

This essay attempts to assess William Wilde as a social historian. It examines some of his contributions to the discipline of history and looks particularly at 'The food of the Irish', which was published in the Dublin University Magazine in February 1854.

75 FR 39910 - Prince William Sound Resource Advisory Committee; Meeting

Science.gov (United States)

2010-07-13

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee; Meeting AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource..., Anchorage, Alaska 99503. Send written comments to Prince William Sound Resource Advisory Committee, c/o USDA...

A new case of keratoconus associated with Williams-Beuren syndrome.

Science.gov (United States)

Viana, Melissa Machado; Frasson, Maria; Leão, Letícia Lima; Stofanko, Martin; Gonçalves-Dornelas, Higgor; Cunha, Pricila da Silva; de Aguiar, Marcos José Burle

2013-09-01

Williams-Beuren syndrome is a multisystemic genetic disorder caused by a contiguous gene deletion at 7q11.23. Keratoconus is a complex disease and it is suspected to have a genetic origin, although the specific gene responsible for keratoconus has not been identified. Although there are several ocular features in Williams-Beuren syndrome, keratoconus is not regularly described as part of this syndrome. To report a new patient with keratoconus and Williams-Beuren syndrome. This is the third case of an association between Williams-Beuren syndrome and keratoconus. The authors believe that the Williams-Beuren syndrome chromosome region can be a possible target for further investigation as the genetic basis of keratoconus.

Epistemologia pragmatyczna Michaela Williamsa (PRAGMATIST EPISTEMOLOGY BY MICHAEL WILLIAMS

Directory of Open Access Journals (Sweden)

Renata Ziemińska

2007-06-01

Full Text Available The article presents three main elements of Williams' epistemology: the concept of knowledge, the problem of skepticism and the concept of truth. Williams takes knowledge not as pure descriptive but partly normative concept (to know is to be engaged and entitled. He rejects the demonstrative conception of knowledge (knowledge is infallible and prefers the fallibilist conception of knowledge (knowledge is uncertain and fallible. Williams is good at bringing skeptical presuppositions to light: the demonstrative conception of knowledge and the conception of justification with Prior Grounding Requirement, epistemological realism and priority for internal knowledge. He rightly observes that when we change that presuppositions (skeptic's context, knowledge does exist. However, Williams-fallibilist is close to a skeptic: they both agree that our beliefs are uncertain. The difference is only whether some of our beliefs deserve to be called knowledge. The most important worries concern Williams' concept of truth (deflationary pragmatism. According to Williams truth has no nature and it is not a goal of inquiry. However, if truth is not a goal, we can hardly understand the previous discussion with skepticism and the defense of rationality.

33 CFR 161.60 - Vessel Traffic Service Prince William Sound.

Science.gov (United States)

2010-07-01

... William Sound. 161.60 Section 161.60 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND... Movement Reporting System Areas and Reporting Points § 161.60 Vessel Traffic Service Prince William Sound... Cape Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between...

Parent insights into atypicalities of social approach behaviour in Williams syndrome.

Science.gov (United States)

Lough, E; Rodgers, J; Janes, E; Little, K; Riby, D M

2016-11-01

Individuals with Williams syndrome have been reported to show high levels of social interest and a desire to interact with others irrespective of their familiarity. This high social motivation, when combined with reduced intellectual capacity and a profile of atypical social behaviour, is important in terms of social vulnerability of individuals with the disorder. Therefore, social approach to unfamiliar people and the role of this behaviour within the Williams syndrome (WS) social phenotype warrant further research to inform social skills' intervention design. The current study used parent interviews (n = 21) to probe aspects of social behaviour and interactions with strangers, as well as the impact of such behaviour on the family. Using thematic analysis, it was possible to explore themes that emerged from the interviews, offering qualitatively rich insight into the variability of social approach behaviour in WS. Thematic analysis confirmed a significant desire to interact with strangers as well as a lack of awareness of appropriate social boundaries. However, parental reports about their child's social approach behaviour varied considerably. The within-syndrome variability of the sample was emphasised in parental reports of their child's personality characteristics (e.g. levels of impulsiveness), as well as the level of parental supervision employed. These in-depth parent insights can help target the needs of individuals with WS and emphasise that an individual approach to intervention will be essential because of the heterogeneity of the WS social profile. © 2016 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

William Whewell, Galileo, and reconceptualizing the history of science and religion.

Science.gov (United States)

Wilson, David B

2011-12-20

This paper advocates a reconceptualization of the history of science and religion. It is an approach to the subject that would aid research by historians of science as well as their message to others, both academic and non-academic. The approach is perfectly illustrated by the life and ideas of William Whewell and Galileo.

Discoverers of the universe William and Caroline Herschel

CERN Document Server

Hoskin, Michael

2011-01-01

Discoverers of the Universe tells the gripping story of William Herschel, the brilliant, fiercely ambitious, emotionally complex musician and composer who became court astronomer to Britain's King George III, and of William's sister, Caroline, who assisted him in his observations of the night sky and became an accomplished astronomer in her own right. Together, they transformed our view of the universe from the unchanging, mechanical creation of Newton's clockmaker god to the ever-evolving, incredibly dynamic cosmos that it truly is. William was in his forties when his amateur observations usi

False Belief Attribution in Children with Williams Syndrome: The Answer Is in The Emotion

Science.gov (United States)

Campos, R.; Martínez-Castilla, P.; Sotillo, M.

2017-01-01

Background: Individuals with Williams syndrome (WS) show difficulties in attributing false beliefs, whereas they are better at attributing emotions. This study examines whether being asked about the emotion linked to a false belief, instead of explicitly about the belief, facilitates performance on theory of mind (ToM) tasks. Method: Thirty…

The William and Flora Hewlett Foundation | IDRC - International ...

International Development Research Centre (IDRC) Digital Library (Canada)

The William and Flora Hewlett Foundation. The William and Flora Hewlett Foundation. http://www.hewlett.org/. Think Tank Initiative. This initiative is creating high-quality independent research and policy institutions throughout the developing world. View more. Think Tank Initiative. Growth and Economic Opportunities for ...

The Unrequited Love as Reflected in William Blake's “Love's Secret”

OpenAIRE

Risthania, Olivia Fergie

2014-01-01

The purpose of this final academic paper is to describe the love story which tells sadness in William Blake's “Love's Secret”. The writer adopts Erich Fromm's theory of unrequited love from The Art of Loving. This final academic paper concerns intrinsic and extrinsic side of the poem. In the intrinsic side, the writer discusses about the existing diction and figurative language such as denotation and connotation and imagery to understand the true meaning of the poem. The extrinsic side, the w...

34 CFR 685.100 - The William D. Ford Federal Direct Loan Program.

Science.gov (United States)

2010-07-01

... 34 Education 3 2010-07-01 2010-07-01 false The William D. Ford Federal Direct Loan Program. 685...) OFFICE OF POSTSECONDARY EDUCATION, DEPARTMENT OF EDUCATION WILLIAM D. FORD FEDERAL DIRECT LOAN PROGRAM Purpose and Scope § 685.100 The William D. Ford Federal Direct Loan Program. (a) Under the William D. Ford...

Congenital heart defects in Williams syndrome.

Science.gov (United States)

Yuan, Shi-Min

2017-01-01

Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.

Recent paleoseismicity record in Prince William Sound, Alaska, USA

Science.gov (United States)

Kuehl, Steven A.; Miller, Eric J.; Marshall, Nicole R.; Dellapenna, Timothy M.

2017-12-01

Sedimentological and geochemical investigation of sediment cores collected in the deep (>400 m) central basin of Prince William Sound, along with geochemical fingerprinting of sediment source areas, are used to identify earthquake-generated sediment gravity flows. Prince William Sound receives sediment from two distinct sources: from offshore (primarily Copper River) through Hinchinbrook Inlet, and from sources within the Sound (primarily Columbia Glacier). These sources are found to have diagnostic elemental ratios indicative of provenance; Copper River Basin sediments were significantly higher in Sr/Pb and Cu/Pb, whereas Prince William Sound sediments were significantly higher in K/Ca and Rb/Sr. Within the past century, sediment gravity flows deposited within the deep central channel of Prince William Sound have robust geochemical (provenance) signatures that can be correlated with known moderate to large earthquakes in the region. Given the thick Holocene sequence in the Sound ( 200 m) and correspondingly high sedimentation rates (>1 cm year-1), this relationship suggests that sediments within the central basin of Prince William Sound may contain an extraordinary high-resolution record of paleoseismicity in the region.

"I Had Made a Mistake": William H. Kilpatrick and the Project Method

Science.gov (United States)

Knoll, Michael

2012-01-01

Background/Context: William H. Kilpatrick is known worldwide as "Mr. Project Method." Despite considerable scholarship by Lawrence A. Cremin, Herbert M. Kliebard, Milton A. Bleeke, John A. Beineke, and others, the origin of Kilpatrick's celebrated paper of 1918 has never been explored in depth and its historical context. Focus of Study:…

76 FR 69760 - Agency Information Collection Activities: The William T. Pecora Award Application and Nomination...

Science.gov (United States)

2011-11-09

... collection of nominations for the William T. Pecora Award. As part of our continuing efforts to reduce... individuals or groups working in the field of remote sensing of the earth. National and international... Burden Hours: 200 hours. We estimate the public reporting burden averages 10 hours per response...

William Healy, M.D., Father of the American Child Guidance Movement

Science.gov (United States)

1981-08-01

courts, was another important development, starting in 1899. Meyer, Sigmund Freud , and Healy all contributed to the next step, which coupled the...Dummer Papers lists 454 correspondents. The prominent psychiatrists include: Franz Alexander, Trigant Burrow, Havelock Ellis, Flanders Dunbar, Sigmund ... Freud , Roy Grinker, William Healy, Karen Homey, Marion E. Kenworthy, Lawson Lowrey, Julse Masserman, Karl Menninger, Adolf Meyer, Smith Ely Jelliffe

Emotional Responsivity in Young Children with Williams Syndrome

Science.gov (United States)

Fidler, Debbie J.; Hepburn, Susan L.; Most, David E.; Philofsky, Amy; Rogers, Sally J.

2007-01-01

The hypothesis that young children with Williams syndrome show higher rates of emotional responsivity relative to other children with developmental disabilities was explored. Performance of 23 young children with Williams syndrome and 30 MA-matched children with developmental disabilities of nonspecific etiologies was compared on an adaptation of…

Malformations vasculaires au cours du syndrome de Williams ...

African Journals Online (AJOL)

Le syndrome de Williams-Beuren est une maladie génétique rare, il associe classiquement une dysmorphie faciale assez spécifique, des malformations cardiovasculaires et un profil neuropsychologique particulier. Nous rapportons les observations de trois enfants atteints du syndrome de Williams-Beuren en insistant ...

William Carlos Williams’ cubism: The sensory dimension

Directory of Open Access Journals (Sweden)

J-L Kruger

1995-05-01

Full Text Available In this article the cubism of the American poet William Carlos Williams is discussed as a product of sensory elements combined with techniques derived from the work of the visual artists associated with this style. Through the study o f a number of poems written in the period between 1917 and 1923 it is shown that Williams employs the cubist intersection of sensory planes in particular to create a sensory dimension that not only renews the traditions and mode of poetry, but also reveals the cubist concern with the defamiliarization and foregrounding of fragments of everyday experiences. Ultimately the article is an attempt to indicate Williams’ incorporation o f a sensual dimension in creating a style that achieves modernist presentation revealing an independence from both traditional literary and visual styles.

The New Forgotten Half and Research Directions to Support Them. Summary and Key Findings. A William T. Grant Foundation Inequality Paper

Science.gov (United States)

Rosenbaum, James; Ahearn, Caitlin; Becker, Kelly; Rosenbaum, Janet

2015-01-01

In 1988, the William T. Grant Foundation issued "The Forgotten Half," the final report of the Foundation's Commission on Youth and America's Future. Focusing on inequality in American society, specifically among non-college-bound 16-24 year olds, the report explored the challenges facing young people and the institutions that serve them.…

Instruction, Curriculum and Society: Iterations Based on the Ideas of William Doll

Science.gov (United States)

Varbelow, Sonja

2012-01-01

This paper explores the relationship between society and school from the point of view of chaos theory with the purpose to understand the deficiencies in teacher education programs and to offer suggestions for their improvement. Based on the ideas of the postmodern curriculum theorist William Doll, it examines the paradigm shifts of world views…

Infantile ictal apneas in a child with williams-beuren syndrome.

Science.gov (United States)

Myers, Kenneth A; McLeod, D Ross; Bello-Espinosa, Luis

2013-02-01

Williams-Beuren syndrome is a genetic disorder rarely associated with seizures. The few described cases of Williams-Beuren syndrome and epilepsy have primarily involved infantile spasms and deletions extending beyond the common deletion region for this disorder. We present the case of a 5-week-old child with ictal apneas and typical Williams-Beuren syndrome deletion. Diagnosis was challenging, because the child had cardiac, respiratory, and gastrointestinal abnormalities typically associated with Williams-Beuren syndrome, which are also associated with cyanotic episodes. The results of interictal electroencephalography were normal, illustrating that prolonged electroencephalography is often essential in evaluation of suspected ictal apneas. Seizure freedom was achieved with carbamazepine. Sudden death is seen in Williams-Beuren syndrome, and this case raises the question whether some of these cases may be related to ictal apneas and could potentially be preventable with appropriate pharmaceutical intervention. Copyright © 2013 Elsevier Inc. All rights reserved.

Northern gas: Williams petrochemical feasibility study

Energy Technology Data Exchange (ETDEWEB)

Chappell, D. [Williams Energy Canada, Calgary, AB (Canada)

2002-07-01

Williams Energy is a company that is involved in the following fields: gas pipelines, exploration and production, midstream, refining, petrochemical, power, and marketing and trading. The author provides an overview of the global and Canadian infrastructure before proceeding to discuss Arctic gas, which is viewed by Williams Energy as necessary. It favors the Alaska Highway route with a consortium for project development. Williams performed a petrochemical study to determine the feasibility of a petrochemical complex utilizing natural gas liquid from an Arctic gas pipeline. The scope of the study encompassed facilities (extraction plant, cracker, polyolefins plant), size (world scale, approximately 2 billion pounds per year), and location (Fairbanks or Alberta). The study led to the following findings: (1) review of several scenarios for both locations, (2) complex to produce two grades of polyethylene, (3) feedstock cost favors Alaska, (4) construction costs lower in Alberta, (5) and the primary market for the Alaska complex would be northeast Asia, while the primary market for the Alberta location would be the United States. It was determined that both options would be viable, however Alberta was favored due to the polyethylene forecast. The challenges still being faced by Williams include low frac spread, market inefficiencies, empress volumes, carbon dioxide, and fuel and electricity cost. Each of these challenges is discussed separately. The author concludes by indicating that incremental ethane is available, carbon dioxide issues need resolution, and Alberta and Alaska are attractive for Arctic ethane petrochemical production. figs.

Celiac disease in patients with Williams-Beuren syndrome.

Science.gov (United States)

Mıhçı, Ercan; Nur, Banu Güzel; Berker-Karaüzüm, Sibel; Yılmaz, Aygen; Artan, Reha

2015-01-01

Celiac disease is an autoimmune, gastrointestinal disorder characterized by intolerance to the dietary grain protein gluten. An increased prevalence of celiac disease has been reported in Down syndrome and Turner syndrome, but there has been only few previous reports with respect to the association of celiac disease in Williams-Beuren syndrome. The aim of this study was to evaluate the frequency of celiac disease in our 24 Williams-Beuren syndrome patients. Gastrointestinal problems and celiac disease symptoms of patients were noted. All patients were analyzed by the titer of tissue transglutaminases IgA and IgG. HLA genotyping and intestinal biopsy was performed to the patients with positive serology. We also performed gluten free diet in the presence of compatible symptoms, serology, HLA genotyping and intestinal biopsy. In our study, two patients had positive tTG antibodies, but only one had positive biopsy finding for celiac disease. The frequency of celiac disease in patients with Williams-Beuren syndrome was estimated as 1/24 (4.1%). Though the number of participants in this study was limited, the results show that the frequency of celiac disease is higher in Williams-Beuren syndrome compared to the general population. We suggest that a high suspicion and testing for celiac disease should be recommended at certain intervals in all cases with Williams-Beuren syndrome to detect the cause of growth retardation and gastrointestinal problems.

Williams Syndrome and 15q Duplication: Coincidence versus Association.

Science.gov (United States)

Khokhar, Aditi; Agarwal, Swashti; Perez-Colon, Sheila

2017-01-01

Williams syndrome is a multisystem disorder caused by contiguous gene deletion in 7q11.23, commonly associated with distinctive facial features, supravalvular aortic stenosis, short stature, idiopathic hypercalcemia, developmental delay, joint laxity, and a friendly personality. The clinical features of 15q11q13 duplication syndrome include autism, mental retardation, ataxia, seizures, developmental delay, and behavioral problems. We report a rare case of a girl with genetically confirmed Williams syndrome and coexisting 15q duplication syndrome. The patient underwent treatment for central precocious puberty and later presented with primary amenorrhea. The karyotype revealed 47,XX,+mar. FISH analysis for the marker chromosome showed partial trisomy/tetrasomy for proximal chromosome 15q (15p13q13). FISH using an ELN -specific probe demonstrated a deletion in the Williams syndrome critical region in 7q11.23. To our knowledge, a coexistence of Williams syndrome and 15q duplication syndrome has not been reported in the literature. Our patient had early pubertal development, which has been described in some patients with Williams syndrome. However, years later after discontinuing gonadotropin-releasing hormone analogue treatment, she developed primary amenorrhea.

John Dewey, William Wirt and the Gary Schools Plan: A Centennial Reappraisal

Science.gov (United States)

Thorburn, Malcolm

2017-01-01

A century on from the height of John Dewey's educational writings and the reputation of the Gary Schools Plan as a model of progressive education, the paper reappraises two key matters: the relationship between John Dewey and William Wirt, the first superintendent of the Gary Schools in Gary Indiana, and the coherence between John Dewey's…

76 FR 44893 - Prince William Sound Resource Advisory Committee

Science.gov (United States)

2011-07-27

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... District, 145 Forest Station Road, Girdwood, AK; Prince Willam Sound Community College, 303 Lowe Street...

33 CFR 165.1704 - Prince William Sound, Alaska-regulated navigation area.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false Prince William Sound, Alaska... District § 165.1704 Prince William Sound, Alaska-regulated navigation area. (a) The following is a... Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between 146°30′ W...

Florence Jessie Mac Williams

Indian Academy of Sciences (India)

CPMGIKAlBGE-340/2003-05. Resonance - January 2005. Licenced to post WPP No.6 RT Nagar Postoffice. Florence Jessie Mac Williams. (1917 - 1990). Registered with Registrar of Newspapers in India vide Regn. No. 66273/96. ISSN 0971-8044. Price per copy: Rs 40.

77 FR 19301 - Prince William Sound Regional Citizens' Advisory Council Charter Renewal

Science.gov (United States)

2012-03-30

... DEPARTMENT OF HOMELAND SECURITY Coast Guard [USCG-2012-0099] Prince William Sound Regional... Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) as an alternative voluntary advisory group for Prince William Sound, Alaska. This certification allows the PWSRCAC to monitor the activities...

Interaction with William Carnall

International Nuclear Information System (INIS)

Judd, Brian R.

2005-01-01

A personal account is given of interaction with William T. Carnall during the period 1977-1988, when I made regular visits to the Argonne National Laboratory to discuss the theoretical background to the spectroscopic work he was carrying out on the lanthanides and actinides

[Association between Williams syndrome and adrenal insufficiency].

Science.gov (United States)

Rchachi, Meryem; Larwanou, Maazou Mahamane; El Ouahabi, Hanan; Ajdi, Farida

2017-01-01

Williams syndrome is a developmental disorder including dysmorphia, cardiovascular malformations and a specific neuropsychological profile together with other associated disorders. We report the case of a 17-year old girl, born of a non-inbred marriage, with Williams syndrome discovered during an assessment of degree of failure to thrive. Its association with primary adrenal insufficiency makes it unique. Diagnosis is confirmed by cytogenetic and molecular analysis. Its management consists of the implementation of treatment for adrenal insufficiency associated with a clinico-biological monitoring.

The Williams syndrome prosociality gene GTF2I mediates oxytocin reactivity and social anxiety in a healthy population.

Science.gov (United States)

Procyshyn, Tanya L; Spence, Jason; Read, Silven; Watson, Neil V; Crespi, Bernard J

2017-04-01

The neurohormone oxytocin plays a central role in human social behaviour and cognition, and oxytocin dysregulation may contribute to psychiatric disorders. However, genetic factors influencing individual variation in the oxytocinergic system remain poorly understood. We genotyped 169 healthy adults for a functional polymorphism in GTF2I ( general transcription factor II-I ), a gene associated with high prosociality and reduced social anxiety in Williams syndrome, a condition reported to involve high oxytocin levels and reactivity. Participants' salivary oxytocin levels were measured before and after watching a validated empathy-inducing video. Oxytocin reactivity, defined as pre- to post-video percentage change in salivary oxytocin, varied substantially and significantly between individuals with different GTF2I genotypes, with, additionally, a trend towards an interaction between genotype and sex. Individuals with more oxytocin-reactive genotypes also reported significantly lower social anxiety. These findings suggest a model whereby GTF2I has a continuum of effects on human sociality, from the extreme social phenotypes and oxytocin dysregulation associated with gene deletion in Williams syndrome, to individual differences in oxytocin reactivity and sociality associated with common polymorphisms in healthy populations. © 2017 The Author(s).

Perceptual learning in Williams syndrome: looking beyond averages.

Directory of Open Access Journals (Sweden)

Patricia Gervan

Full Text Available Williams Syndrome is a genetically determined neurodevelopmental disorder characterized by an uneven cognitive profile and surprisingly large neurobehavioral differences among individuals. Previous studies have already shown different forms of memory deficiencies and learning difficulties in WS. Here we studied the capacity of WS subjects to improve their performance in a basic visual task. We employed a contour integration paradigm that addresses occipital visual function, and analyzed the initial (i.e. baseline and after-learning performance of WS individuals. Instead of pooling the very inhomogeneous results of WS subjects together, we evaluated individual performance by expressing it in terms of the deviation from the average performance of the group of typically developing subjects of similar age. This approach helped us to reveal information about the possible origins of poor performance of WS subjects in contour integration. Although the majority of WS individuals showed both reduced baseline and reduced learning performance, individual analysis also revealed a dissociation between baseline and learning capacity in several WS subjects. In spite of impaired initial contour integration performance, some WS individuals presented learning capacity comparable to learning in the typically developing population, and vice versa, poor learning was also observed in subjects with high initial performance levels. These data indicate a dissociation between factors determining initial performance and perceptual learning.

Anxiety in Williams Syndrome: The Role of Social Behaviour, Executive Functions and Change over Time

Science.gov (United States)

Ng-Cordell, Elise; Hanley, Mary; Kelly, Alyssa; Riby, Deborah M.

2018-01-01

Anxiety is a prevalent mental health issue for individuals with Williams syndrome (WS). Relatively little is known about the developmental course of anxiety, or how it links with core features of WS, namely social and executive functioning (EF). In this study, parent-reports of anxiety were compared across a 4-year period (N = 17), and links…

Williams' paradox and the role of phenotypic plasticity in sexual systems.

Science.gov (United States)

Leonard, Janet L

2013-10-01

As George Williams pointed out in 1975, although evolutionary explanations, based on selection acting on individuals, have been developed for the advantages of simultaneous hermaphroditism, sequential hermaphroditism and gonochorism, none of these evolutionary explanations adequately explains the current distribution of these sexual systems within the Metazoa (Williams' Paradox). As Williams further pointed out, the current distribution of sexual systems is explained largely by phylogeny. Since 1975, we have made a great deal of empirical and theoretical progress in understanding sexual systems. However, we still lack a theory that explains the current distribution of sexual systems in animals and we do not understand the evolutionary transitions between hermaphroditism and gonochorism. Empirical data, collected over the past 40 years, demonstrate that gender may have more phenotypic plasticity than was previously realized. We know that not only sequential hermaphrodites, but also simultaneous hermaphrodites have phenotypic plasticity that alters sex allocation in response to social and environmental conditions. A focus on phenotypic plasticity suggests that one sees a continuum in animals between genetically determined gonochorism on the one hand and simultaneous hermaphroditism on the other, with various types of sequential hermaphroditism and environmental sex determination as points along the spectrum. Here I suggest that perhaps the reason we have been unable to resolve Williams' Paradox is because the problem was not correctly framed. First, because, for example, simultaneous hermaphroditism provides reproductive assurance or dioecy ensures outcrossing does not mean that there are no other evolutionary paths that can provide adaptive responses to those selective pressures. Second, perhaps the question we need to ask is: What selective forces favor increased versus reduced phenotypic plasticity in gender expression? It is time to begin to look at the question

Neural correlates of amusia in williams syndrome.

Science.gov (United States)

Lense, Miriam D; Dankner, Nathan; Pryweller, Jennifer R; Thornton-Wells, Tricia A; Dykens, Elisabeth M

2014-11-21

Congenital amusia is defined by marked deficits in pitch perception and production. Though historically examined only in otherwise typically developing (TD) populations, amusia has recently been documented in Williams syndrome (WS), a genetic, neurodevelopmental disorder with a unique auditory phenotype including auditory sensitivities and increased emotional responsiveness to music but variable musical skill. The current study used structural T1-weighted magnetic resonance imaging and diffusion tensor imaging to examine neural correlates of amusia in 17 individuals with WS (4 of whom met criteria for amusia). Consistent with findings from TD amusics, amusia in WS was associated with decreased fractional anisotropy (FA) in the right superior longitudinal fasciculus (SLF). The relationship between amusia and FA in the inferior component of the SLF was particularly robust, withstanding corrections for cognitive functioning, auditory sensitivities, or musical training. Though the number of individuals with amusia in the study is small, results add to evidence for the role of fronto-temporal disconnectivity in congenital amusia and suggest that novel populations with developmental differences can provide a window into understanding gene-brain-behavior relationships that underlie musical behaviors.

The Influence of the Doctor’s Mind Style in Spring and All on William Carlos Williams’s Poetry Creation

Directory of Open Access Journals (Sweden)

Meiling Fu

2017-05-01

Full Text Available This article intends to investigate the doctor’s mind style on William Carlos Williams in his collection of poems Spring and All. Research shows that Williams’s doctor’s life makes him shape a different view of the world. He always observes something difficult or challenging at first and notices something easy and comfortable at last. This paper concludes that Williams’s doctor’s mind style exerts influence on his creation of poetry both in content and in form.

Pragmatic Language Assessment in Williams Syndrome: A Comparison of the Test of Pragmatic Language--2 and the Children's Communication Checklist--2

Science.gov (United States)

Hoffmann, Anne; Martens, Marilee A.; Fox, Robert; Rabidoux, Paula; Andridge, Rebecca

2013-01-01

Purpose: Individuals with Williams syndrome (WS) are recognized as having a strong desire for social relationships, yet many of them have difficulty forming and maintaining peer relationships. One cause may be impairments in pragmatic language. The current study compared the assessment of pragmatic language skills in individuals with WS using the…

3D PATTERN OF BRAIN ABNORMALITIES IN WILLIAMS SYNDROME VISUALIZED USING TENSOR-BASED MORPHOMETRY

OpenAIRE

Chiang, Ming-Chang; Reiss, Allan L.; Lee, Agatha D.; Bellugi, Ursula; Galaburda, Albert M.; Korenberg, Julie R.; Mills, Debra L.; Toga, Arthur W.; Thompson, Paul M.

2007-01-01

Williams syndrome (WS) is a neurodevelopmental disorder associated with deletion of ~20 contiguous genes in chromosome band 7q11.23. Individuals with WS exhibit mild to moderate mental retardation, but are relatively more proficient in specific language and musical abilities. We used tensor-based morphometry (TBM) to visualize the complex pattern of gray/white matter reductions in WS, based on fluid registration of structural brain images.

Genes, language, and the nature of scientific explanations: the case of Williams syndrome.

Science.gov (United States)

Musolino, Julien; Landau, Barbara

2012-01-01

In this article, we discuss two experiments of nature and their implications for the sciences of the mind. The first, Williams syndrome, bears on one of cognitive science's holy grails: the possibility of unravelling the causal chain between genes and cognition. We sketch the outline of a general framework to study the relationship between genes and cognition, focusing as our case study on the development of language in individuals with Williams syndrome. Our approach emphasizes the role of three key ingredients: the need to specify a clear level of analysis, the need to provide a theoretical account of the relevant cognitive structure at that level, and the importance of the (typical) developmental process itself. The promise offered by the case of Williams syndrome has also given rise to two strongly conflicting theoretical approaches-modularity and neuroconstructivism-themselves offshoots of a perennial debate between nativism and empiricism. We apply our framework to explore the tension created by these two conflicting perspectives. To this end, we discuss a second experiment of nature, which allows us to compare the two competing perspectives in what comes close to a controlled experimental setting. From this comparison, we conclude that the "meaningful debate assumption", a widespread assumption suggesting that neuroconstructivism and modularity address the same questions and represent genuine theoretical alternatives, rests on a fallacy.

Discovering Structure in Auditory Input: Evidence from Williams Syndrome

Science.gov (United States)

Elsabbagh, Mayada; Cohen, Henri; Karmiloff-Smith, Annette

2010-01-01

We examined auditory perception in Williams syndrome by investigating strategies used in organizing sound patterns into coherent units. In Experiment 1, we investigated the streaming of sound sequences into perceptual units, on the basis of pitch cues, in a group of children and adults with Williams syndrome compared to typical controls. We showed…

Dental management of patient with Williams Syndrome - A case report.

Science.gov (United States)

Wong, Daniel; Ramachandra, Srinivas Sulugodu; Singh, Ashish Kumar

2015-01-01

Williams syndrome is a multisystemic rare genetic disorder caused by deletion of 26-28 genes in the long arm of chromosome 7. It is characterized by developmental and physical abnormalities including congenital cardiovascular abnormalities, mental retardation, neurological features, growth deficiency, genitourinary manifestations, gastrointestinal problems, musculoskeletal problems, unique behavioral characteristics, and dental problems. Dental abnormalities include malocclusion, hypodontia, malformed teeth, taurodontism, pulp stones, increased space between teeth, enamel hypoplasia, and high prevalence of dental caries. Authors report a 17-year-old female patient with underlying Williams syndrome. Oral features and problems seen in the patient are listed. Malocclusion and screwdriver shaped teeth were noticed. Generalized widening of the periodontal ligament space with vital teeth was seen. This finding has not been reported in cases of Williams syndrome earlier. Precautions taken during dental treatment in patients with Williams syndrome are also discussed.

Educational "Goodwill": Measuring the Intangible Assets at Highly Selective Private Colleges and Universities. NBER Working Paper No. 17412

Science.gov (United States)

Nurnberg, Peter; Schapiro, Morton; Zimmerman, David

2011-01-01

In this paper we utilize data on the head-to-head loss rate for students accepted at Williams College, but who opt to enroll elsewhere. For example, we employ data that measure the fraction of students admitted to Williams and to Amherst (or Harvard or Yale, etc.) but who opt to attend Amherst (or Harvard or Yale, etc.) instead of Williams. We…

William Graham Brooke (1835-1907): Advocate of Girls' Superior Schooling in Nineteenth-Century Ireland

Science.gov (United States)

McCormack, Christopher F.

2015-01-01

This paper examines the role of William Graham Brooke as advocate of women's higher education and access to university. His work as advocate is considered against the religious, political, social and economic backdrop of late nineteenth century Ireland. A barrister, as Clerk in the Lord Chancellor's office, he was centrally involved in the…

The William Houston Medal of the Royal College of Surgeons of Edinburgh 2002.

Science.gov (United States)

Teague, A M

2004-06-01

The William Houston medal is a prestigious prize awarded to the individual achieving the most outstanding examination performance at the Membership in Orthodontics examination for the Royal College of Surgeons of Edinburgh. Five clinical cases treated by the candidate are presented as part of the final examination; two of these cases are described below. The first a Class III malocclusion, and the second a Class II division 1 malocclusion, were both treated by orthodontic camouflage.

75 FR 16159 - Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) Charter Renewal

Science.gov (United States)

2010-03-31

... DEPARTMENT OF HOMELAND SECURITY Coast Guard [USCG-2010-0121] Prince William Sound Regional... the Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) as an alternative voluntary advisory group for Prince William Sound, Alaska. This certification allows the PWSRCAC to monitor the...

Auditory cortical volumes and musical ability in Williams syndrome.

Science.gov (United States)

Martens, Marilee A; Reutens, David C; Wilson, Sarah J

2010-07-01

Individuals with Williams syndrome (WS) have been shown to have atypical morphology in the auditory cortex, an area associated with aspects of musicality. Some individuals with WS have demonstrated specific musical abilities, despite intellectual delays. Primary auditory cortex and planum temporale volumes were manually segmented in 25 individuals with WS and 25 control participants, and the participants also underwent testing of musical abilities. Left and right planum temporale volumes were significantly larger in the participants with WS than in controls, with no significant difference noted between groups in planum temporale asymmetry or primary auditory cortical volumes. Left planum temporale volume was significantly increased in a subgroup of the participants with WS who demonstrated specific musical strengths, as compared to the remaining WS participants, and was highly correlated with scores on a musical task. These findings suggest that differences in musical ability within WS may be in part associated with variability in the left auditory cortical region, providing further evidence of cognitive and neuroanatomical heterogeneity within this syndrome. Copyright (c) 2010 Elsevier Ltd. All rights reserved.

Anderson, Prof. Basil Williams

Indian Academy of Sciences (India)

Home; Fellowship. Fellow Profile. Elected: 1964 Honorary. Anderson, Prof. Basil Williams. Date of birth: 3 July 1901. Date of death: 24 February 1984. YouTube; Twitter; Facebook; Blog. Academy News. IAS Logo. 29th Mid-year meeting. Posted on 19 January 2018. The 29th Mid-year meeting of the Academy will be held ...

Series, Prof. George William

Indian Academy of Sciences (India)

Home; Fellowship. Fellow Profile. Elected: 1984 Honorary. Series, Prof. George William. Date of birth: 22 February 1920. Date of death: 2 January 1995. YouTube; Twitter; Facebook; Blog. Academy News. IAS Logo. 29th Mid-year meeting. Posted on 19 January 2018. The 29th Mid-year meeting of the Academy will be held ...

Route-Learning Strategies in Typical and Atypical Development; Eye Tracking Reveals Atypical Landmark Selection in Williams Syndrome

Science.gov (United States)

Farran, E. K.; Formby, S.; Daniyal, F.; Holmes, T.; Van Herwegen, J.

2016-01-01

Background: Successful navigation is crucial to everyday life. Individuals with Williams syndrome (WS) have impaired spatial abilities. This includes a deficit in spatial navigation abilities such as learning the route from A to B. To-date, to determine whether participants attend to landmarks when learning a route, landmark recall tasks have been…

Effect of Musical Experience on Verbal Memory in Williams Syndrome: Evidence from a Novel Word Learning Task

Science.gov (United States)

Martens, Marilee A.; Jungers, Melissa K.; Steele, Anita L.

2011-01-01

Williams syndrome (WS) is a neurogenetic developmental disorder characterized by an increased affinity for music, deficits in verbal memory, and atypical brain development. Music has been shown to improve verbal memory in typical individuals as well as those with learning difficulties, but no studies have examined this relationship in WS. The aim…

33 CFR 167.1701 - In Prince William Sound: Precautionary areas.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false In Prince William Sound: Precautionary areas. 167.1701 Section 167.1701 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF... Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1701 In Prince William Sound...

A MacWilliams Identity for Convolutional Codes : The General Case

NARCIS (Netherlands)

Gluesing-Luerssen, Heide; Schneider, Gert

A MacWilliams Identity for convolutional codes will be established. It makes use of the weight adjacency matrices of the code and its dual, based on state space realizations (the controller canonical form) of the codes in question. The MacWilliams Identity applies to various notions of duality

Speech Timing and Verbal Short-Term Memory: Evidence for Contrasting Deficits in Down Syndrome and Williams Syndrome

Science.gov (United States)

Jarrold, Christopher; Cowan, Nelson; Hewes, Alexa K.; Riby, Deborah M.

2004-01-01

This study explored the degree of verbal short-term memory deficit among individuals with Down syndrome and Williams syndrome, and the extent to which any such impairment could be accounted for by a relative slowing of rehearsal and output processes. Measures of serial recall and detailed assessments of speeded articulation for short and long…

Probed Serial Recall in Williams Syndrome: Lexical Influences on Phonological Short-Term Memory

Science.gov (United States)

Brock, Jan; McCormack, Teresa; Boucher, Jill

2005-01-01

Williams syndrome is a genetic disorder that, it has been claimed, results in an unusual pattern of linguistic strengths and weaknesses. The current study investigated the hypothesis that there is a reduced influence of lexical knowledge on phonological short-term memory in Williams syndrome. Fourteen children with Williams syndrome and 2…

78 FR 28953 - William D. Ford Federal Direct Loan Program

Science.gov (United States)

2013-05-16

.... Ford Federal Direct Loan Program; Interim Final Rule #0;#0;Federal Register / Vol. 78, No. 95... [Docket ID ED-2013-OPE-0066] RIN 1840-AD13 William D. Ford Federal Direct Loan Program AGENCY: Office of... Secretary amends the William D. Ford Federal Direct Loan Program (Direct Loan Program) regulations to...

Pursuing the Panderer: An Analysis of "United States v. Williams"

Science.gov (United States)

McGrain, Patrick N.; Moore, Jennifer L.

2010-01-01

In May 2008, the Supreme Court addressed whether the government can regulate the ownership and distribution of virtual child pornography. "U.S. v. Williams" marked the first time the Court directly addressed the concept of pandering virtual child pornography. This article examines the Court's decision in "U.S. v. Williams" and…

Neural Correlates of Amusia in Williams Syndrome

Directory of Open Access Journals (Sweden)

Miriam D. Lense

2014-11-01

Full Text Available Congenital amusia is defined by marked deficits in pitch perception and production. Though historically examined only in otherwise typically developing (TD populations, amusia has recently been documented in Williams syndrome (WS, a genetic, neurodevelopmental disorder with a unique auditory phenotype including auditory sensitivities and increased emotional responsiveness to music but variable musical skill. The current study used structural T1-weighted magnetic resonance imaging and diffusion tensor imaging to examine neural correlates of amusia in 17 individuals with WS (4 of whom met criteria for amusia. Consistent with findings from TD amusics, amusia in WS was associated with decreased fractional anisotropy (FA in the right superior longitudinal fasciculus (SLF. The relationship between amusia and FA in the inferior component of the SLF was particularly robust, withstanding corrections for cognitive functioning, auditory sensitivities, or musical training. Though the number of individuals with amusia in the study is small, results add to evidence for the role of fronto-temporal disconnectivity in congenital amusia and suggest that novel populations with developmental differences can provide a window into understanding gene-brain-behavior relationships that underlie musical behaviors.

Prince William Sound, Alaska ESI: INDEX

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Fiction and Conviction | Blackburn | Philosophical Papers

African Journals Online (AJOL)

I claim that there is nothing so unusual in the interleaving of myth or fiction and history that Williams finds in Herodotus. I also reflect on the difficulty of separating acceptance of truth from acceptance of myth, metaphor, and model, not only in history but also in science. Philosophical Papers Vol.32(3) 2003: 243-260 ...

76 FR 1187 - Application for Recertification of Prince William Sound Regional Citizens' Advisory Council

Science.gov (United States)

2011-01-07

... Prince William Sound Regional Citizens' Advisory Council AGENCY: Coast Guard, DHS. ACTION: Notice of... on, the application for recertification submitted by the Prince William Sound Regional Citizen's... advisory group in lieu of a Regional Citizens' Advisory Council for Prince William Sound, Alaska. This...

Shoreline ecology program for Prince William Sound, Alaska, following the Exxon Valdez oil spill. Part 3: Biology

International Nuclear Information System (INIS)

Gilfillan, E.S.; Page, D.S.; Harner, E.J.; Boehm, P.D.

1995-01-01

This study describes the biological results of a comprehensive shoreline ecology program designed to assess ecological recovery in Prince William Sound following the Exxon Valdez oil spill on march 24, 1989. The program is an application of the ''Sediment Quality Triad'' approach, combining chemical, toxicological, and biological measurements. The study was designed so that results could be extrapolated to the entire spill zone in Prince William Sound. The spill affected four major shoreline habitat types in Prince William Sound: pebble/gravel, boulder/cobble, sheltered bedrock, and exposed bedrock. The study design had two components: (1) one-time stratified random sampling at 64 sites representing four habitats and four oiling levels (including unoiled reference sites) and (2) periodic sampling at 12 nonrandomly chosen sites that included some of the most heavily oiled locations in the sound. Biological communities on rock surfaces and in intertidal and shallow subtidal sediments were analyzed for differences resulting from to oiling in each of 16 habitat/tide zone combinations. Statistical methods included univariate analyses of individual species abundances and community parameter variables (total abundance, species richness, and Shannon diversity), and multivariate correspondence analysis of community structure. 58 refs., 13 figs., 9 tabs

Dreamers in dialogue: evolution, sex and gender in the utopian visions of William Morris and William Henry Hudson

Directory of Open Access Journals (Sweden)

Caterina Novák

2013-12-01

Full Text Available The aim of this article is to explore the parallels between two late-nineteenth-century utopias,William Henry Hudsons A Crystal Age (1882 and William Morriss News from Nowhere (1891. Itaims to explore how these two works respond to the transition from a kinetic to a static conception ofutopia that under pressure from evolutionary and feminist discourses took place during the period.Particular focus lies on the way in which this is negotiated through the depiction of evolution, sexuality,and gender roles in the respective novels, and how the depiction of these disruptive elements may workas a means of ensuring the readers active engagement in political, intellectual and emotional terms.

Spazio psichico e organizzazione sociale in Neuromancer (1984 di William Gibson

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Tommaso Meozzi

2013-12-01

Full Text Available Neuromancer (1984 by William Gibson is a central text in the cyberpunk genre. This article analyzes how, in this book, computing is a metaphor used to represent some characteristics of the psyche and also a social organization not subject to review by a single person. Memory is individual, embodied, and changes continuously. Its transposition in external media has the price of a symbolic death for the subject. At the same time the impersonality of the social machines becomes the object of a psychological projection by the individual, and takes on anthropomorphic forms. The “Matrix” is the space of “virtual” relationships (in the sense of computing. Through simulative processes that take place in real time, it’s possible to make an illusory fusion of the subject with the social body which I, however, linked to a repression of the deeper drives, affective and individual experience. The story ends when the protagonist becomes able to distinguish between external reality and psychic life.

A case of William's syndrome associated peripheral pulmonary arterial stenosis

International Nuclear Information System (INIS)

Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan

1988-01-01

William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures

A MacWilliams Identity for Convolutional Codes: The General Case

OpenAIRE

Gluesing-Luerssen, Heide; Schneider, Gert

2008-01-01

A MacWilliams Identity for convolutional codes will be established. It makes use of the weight adjacency matrices of the code and its dual, based on state space realizations (the controller canonical form) of the codes in question. The MacWilliams Identity applies to various notions of duality appearing in the literature on convolutional coding theory.

Capacity to improve fine motor skills in Williams syndrome.

Science.gov (United States)

Berencsi, A; Gombos, F; Kovács, I

2016-10-01

Individuals with Williams syndrome (WS) are known to have difficulties in carrying out fine motor movements; however, a detailed behavioural profile of WS in this domain is still missing. It is also unknown how great the capacity to improve these skills with focused and extensive practice is. We studied initial performance and learning capacity in a sequential finger tapping (FT) task in WS and in typical development. Improvement in the FT task has been shown to be sleep dependent. WS subjects participating in the current study have also participated in earlier polysomnography studies, although not directly related to learning. WS participants presented with great individual variability. In addition to generally poor initial performance, learning capacity was also greatly limited in WS. We found indications that reduced sleep efficiency might contribute to this limitation. Estimating motor learning capacity and the depth of sleep disorder in a larger sample of WS individuals might reveal important relationships between sleep and learning, and contribute to efficient intervention methods improving skill acquisition in WS. © 2016 The Authors. Journal of Intellectual Disability Research published by MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

Duncan F. Gregory, William Walton and the development of British algebra: 'algebraical geometry', 'geometrical algebra', abstraction.

Science.gov (United States)

Verburgt, Lukas M

2016-01-01

This paper provides a detailed account of the period of the complex history of British algebra and geometry between the publication of George Peacock's Treatise on Algebra in 1830 and William Rowan Hamilton's paper on quaternions of 1843. During these years, Duncan Farquharson Gregory and William Walton published several contributions on 'algebraical geometry' and 'geometrical algebra' in the Cambridge Mathematical Journal. These contributions enabled them not only to generalize Peacock's symbolical algebra on the basis of geometrical considerations, but also to initiate the attempts to question the status of Euclidean space as the arbiter of valid geometrical interpretations. At the same time, Gregory and Walton were bound by the limits of symbolical algebra that they themselves made explicit; their work was not and could not be the 'abstract algebra' and 'abstract geometry' of figures such as Hamilton and Cayley. The central argument of the paper is that an understanding of the contributions to 'algebraical geometry' and 'geometrical algebra' of the second generation of 'scientific' symbolical algebraists is essential for a satisfactory explanation of the radical transition from symbolical to abstract algebra that took place in British mathematics in the 1830s-1840s.

The Possibility of Love Explored Through the Poetry of William Wordsworth

Directory of Open Access Journals (Sweden)

Kathleen O'Dwyer

2008-10-01

Full Text Available This paper addresses the poetry of William Wordsworth as a significant expression of literary romanticism. It argues for a more comprehensive understanding of the tenets of romantic poetry, and consequently for a greater acknowledgement of its contribution to human knowledge, human understanding and human development than has hitherto been recognized. In particular, the paper explores Wordsworthrsquo;s reflections on the role of love in human living, the obstacles to the experience of love and the essential necessity of love in human flourishing. The concentration on the concept of love enables the promotion of a strong argument in favour of a broader interpretation of romanticism than has been previously been accepted.br /

Comparing the Broad Socio-Cognitive Profile of Youth with Williams Syndrome and 22Q11.2 Deletion Syndrome

Science.gov (United States)

Weisman, O.; Feldman, R.; Burg-Malki, M.; Keren, M.; Geva, R.; Diesendruck, G.; Gothelf, D.

2017-01-01

Background: Numerous studies have assessed the socio-cognitive profile in Williams syndrome (WS) and, independently, in 22q11.2 deletion syndrome (22q11.2DS). Yet, a cross-syndrome comparison of these abilities between individuals with these two syndromes with known social deficits has not been conducted. Methods: Eighty-two children participated…

Anxiety and autonomic response to social-affective stimuli in individuals with Williams syndrome.

Science.gov (United States)

Ng, Rowena; Bellugi, Ursula; Järvinen, Anna

2016-12-01

Williams syndrome (WS) is a genetic condition characterized by an unusual "hypersocial" personality juxtaposed by high anxiety. Recent evidence suggests that autonomic reactivity to affective face stimuli is disorganised in WS, which may contribute to emotion dysregulation and/or social disinhibition. Electrodermal activity (EDA) and mean interbeat interval (IBI) of 25 participants with WS (19 - 57 years old) and 16 typically developing (TD; 17-43 years old) adults were measured during a passive presentation of affective face and voice stimuli. The Beck Anxiety Inventory was administered to examine associations between autonomic reactivity to social-affective stimuli and anxiety symptomatology. The WS group was characterized by higher overall anxiety symptomatology, and poorer anger recognition in social visual and aural stimuli relative to the TD group. No between-group differences emerged in autonomic response patterns. Notably, for participants with WS, increased anxiety was uniquely associated with diminished arousal to angry faces and voices. In contrast, for the TD group, no associations emerged between anxiety and physiological responsivity to social-emotional stimuli. The anxiety associated with WS appears to be intimately related to reduced autonomic arousal to angry social stimuli, which may also be linked to the characteristic social disinhibition. Copyright © 2016. Published by Elsevier Ltd.

Violations of Personal Space in Young People with Autism Spectrum Disorders and Williams Syndrome: Insights from the Social Responsiveness Scale

Science.gov (United States)

Lough, Emma; Hanley, Mary; Rodgers, Jacqui; South, Mikle; Kirk, Hannah; Kennedy, Daniel P.; Riby, Deborah M.

2015-01-01

Interpersonal distance regulation is crucial for successful social interactions. We investigated personal space awareness in Williams syndrome (WS) and autism spectrum disorder (ASD) compared to typical development. Parents reported that individuals with WS and ASD were significantly more likely than those developing typically to invade the…

On Reading Poems: Visual & Verbal Icons in William Carlos Williams' «Landscape With The Fall Of Icarus»

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Irene R. Fairley

1981-09-01

Full Text Available Williams' admiration for Brueghel's landscape is coded in structural and stylistic correspondences between the poem and the painting. Structures in the poem have effects analogous to the use of devices of color, line, foregrounding in the painting. The poem, like the painting, presents a «neutral» scene but subtly insures the reader's involvement. Further, Williams draws a visual statement so that graphic features suggest a global image isomorphic with the motif of descent. Features of the poem, such as line and clause length, syntactic construction, semantic coherence, are discussed as factors that contribute to rapid glancing and increase readability. A study of readers shows their sensitivity to graphic features including the poem's shape, and corroborates the importance of stylistic features.

Audiological follow-up of 24 patients affected by Williams syndrome.

Science.gov (United States)

Barozzi, Stefania; Soi, Daniela; Spreafico, Emanuela; Borghi, Anna; Comiotto, Elisabetta; Gagliardi, Chiara; Selicorni, Angelo; Forti, Stella; Cesarani, Antonio; Brambilla, Daniele

2013-09-01

Williams syndrome is a neurodevelopmental disorder associated with cardiovascular problems, facial abnormalities and several behavioural and neurological disabilities. It is also characterized by some typical audiological features including abnormal sensitivity to sounds, cochlear impairment related to the outer hair cells of the basal turn of the cochlea, and sensorineural or mixed hearing loss, predominantly in the high frequency range. The aim of this report is to describe a follow-up study of auditory function in a cohort of children affected by this syndrome. 24 patients, aged 5-14 years, were tested by means of air/bone conduction pure-tone audiometry, immittance test and transient evoked otoacoustic emissions. They were evaluated again 5 years after the first assessment, and 10 of them underwent a second follow-up examination after a further 5 years. The audiometric results showed hearing loss, defined by a pure tone average >15 dB HL, in 12.5% of the participants. The incidence of hearing loss did not change over the 5-year period and increased to 30% in the patients who underwent the 10-year follow-up. Progressive sensorineural hearing loss was detected in 20% of the patients. A remarkable finding of our study regarded sensorineural hearing impairment in the high frequency range, which increased significantly from 25% to 50% of the participants over the 5-year period. The increase became even more significant in the group of patients who underwent the 10-year follow-up, by which time the majority of them (80%) had developed sensorineural hearing loss. Otoacoustic emissions were found to be absent in a high percentage of patients, thus confirming the cochlear fragility of individuals with Williams syndrome. Our study verified that most of the young Williams syndrome patients had normal hearing sensitivity within the low-middle frequency range, but showed a weakness regarding the high frequencies, the threshold of which worsened significantly over time in

William Harvey, Aristotle and astrology.

Science.gov (United States)

Gregory, Andrew

2014-06-01

In this paper I argue that William Harvey believed in a form of astrology. It has long been known that Harvey employed a macrocosm-microcosm analogy and used alchemical terminology in describing how the two types of blood change into one another. This paper then seeks to examine a further aspect of Harvey in relation to the magical tradition. There is an important corollary to this line of thought, however. This is that while Harvey does have a belief in astrology, it is strongly related to Aristotle's views in this area and is quite restricted and attenuated relative to some contemporary beliefs in astrology. This suggests a more general thesis. While Harvey was amenable to ideas which we associate with the natural magic tradition, those ideas had a very broad range of formulation and there was a limit to how far he would accept them. This limit was largely determined by Harvey's adherence to Aristotle's natural philosophy and his Christian beliefs. I argue that this is also the case in relation to Harvey's use of the macrocosm-microcosm analogy and of alchemical terminology, and, as far as we can rely on the evidence, this informs his attitudes towards witches as well. Understanding Harvey's influences and motives here is important in placing him properly in the context of early seventeenth-century thought.

The proceedings of the 15th professional conference on Williams Syndrome.

Science.gov (United States)

Walton, Jennifer R; Martens, Marilee A; Pober, Barbara R

2017-05-01

Williams Syndrome (WS) is a contiguous gene deletion disorder, caused by the deletion of approximately 26-28 genes from chromosome 7 (7q11.23). Individuals with WS have complex medical, developmental, and behavioral features, requiring multidisciplinary and interdisciplinary collaboration. Guidelines detailing the identification, evaluation, and monitoring of individuals with WS need clarification, especially for primary care providers who are first-line in their management. This report summarizes the proceedings of the 2016 Professional Conference on WS in Columbus, OH. Presentations were directed towards primary care providers and subspecialists, emphasizing evidence-based practices for treating the prevalent medical and behavioral features of WS. Included in this report are findings from a panel of cardiovascular experts discussing three case studies on treatment of hypertension and the use of sedation or anesthesia for non-cardiac procedures. Abstracts from individual expert presenters are included, covering various medical and behavioral topics, and providing updates in management of WS individuals. The following topics were discussed: differences in phenotypes of 7q11.23 deletion versus duplication, growth parameters, endocrine concerns, sleep difficulties, behaviors to monitor, and pharmacological options, the neurodevelopmental profile of WS individuals, and the importance of monitoring medical and behavioral concerns as WS individuals transition to adulthood. © 2017 Wiley Periodicals, Inc.

78 FR 11857 - Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan...

Science.gov (United States)

2013-02-20

...; Comment Request; William D. Ford Federal Direct Loan Program (DL) Regulations AGENCY: Department of... in response to this notice will be considered public records. Title of Collection: William D. Ford.... Abstract: The William D. Ford Federal Direct Loan Program regulations cover areas of program administration...

Rorty, Williams, and Davidson: Skepticism and Metaepistemology

Directory of Open Access Journals (Sweden)

Christopher Ranalli

2013-07-01

Full Text Available We revisit an important exchange on the problem of radical skepticism between Richard Rorty and Michael Williams. In his contribution to this exchange, Rorty defended the kind of transcendental approach to radical skepticism that is offered by Donald Davidson, in contrast to Williams’s Wittgenstein-inspired view. It is argued that the key to evaluating this debate is to understand the particular conception of the radical skeptical problem that is offered in influential work by Barry Stroud, a conception of the skeptical problem which generates metaepistemological ramifications for anti-skeptical theories. In particular, we argue that, contra Williams, Rorty’s view that Davidson was offering a theoretical diagnosis of radical skepticism can be consistently maintained with his transcendental approach.

Autistic disorder in patients with Williams-Beuren syndrome: a reconsideration of the Williams-Beuren syndrome phenotype.

Science.gov (United States)

Tordjman, Sylvie; Anderson, George M; Botbol, Michel; Toutain, Annick; Sarda, Pierre; Carlier, Michèle; Saugier-Veber, Pascale; Baumann, Clarisse; Cohen, David; Lagneaux, Céline; Tabet, Anne-Claude; Verloes, Alain

2012-01-01

Williams-Beuren syndrome (WBS), a rare developmental disorder caused by deletion of contiguous genes at 7q11.23, has been characterized by strengths in socialization (overfriendliness) and communication (excessive talkativeness). WBS has been often considered as the polar opposite behavioral phenotype to autism. Our objective was to better understand the range of phenotypic expression in WBS and the relationship between WBS and autistic disorder. The study was conducted on 9 French individuals aged from 4 to 37 years old with autistic disorder associated with WBS. Behavioral assessments were performed using Autism Diagnostic Interview-Revised (ADI-R) and Autism Diagnostic Observation Schedule (ADOS) scales. Molecular characterization of the WBS critical region was performed by FISH. FISH analysis indicated that all 9 patients displayed the common WBS deletion. All 9 patients met ADI-R and ADOS diagnostic criteria for autism, displaying stereotypies and severe impairments in social interaction and communication (including the absence of expressive language). Additionally, patients showed improvement in social communication over time. The results indicate that comorbid autism and WBS is more frequent than expected and suggest that the common WBS deletion can result in a continuum of social communication impairment, ranging from excessive talkativeness and overfriendliness to absence of verbal language and poor social relationships. Appreciation of the possible co-occurrence of WBS and autism challenges the common view that WBS represents the opposite behavioral phenotype of autism, and might lead to improved recognition of WBS in individuals diagnosed with autism.

The Extent of Working Memory Deficits Associated with Williams Syndrome: Exploration of Verbal and Spatial Domains and Executively Controlled Processes

Science.gov (United States)

Rhodes, Sinead M.; Riby, Deborah M.; Fraser, Emma; Campbell, Lorna Elise

2011-01-01

The present study investigated verbal and spatial working memory (WM) functioning in individuals with the neuro-developmental disorder Williams syndrome (WS) using WM component tasks. While there is strong evidence of WM impairments in WS, previous research has focused on short-term memory and has neglected assessment of executive components of…

Resting-State Functional Connectivity in Individuals with Down Syndrome and Williams Syndrome Compared with Typically Developing Controls.

Science.gov (United States)

Vega, Jennifer N; Hohman, Timothy J; Pryweller, Jennifer R; Dykens, Elisabeth M; Thornton-Wells, Tricia A

2015-10-01

The emergence of resting-state functional connectivity (rsFC) analysis, which examines temporal correlations of low-frequency (syndrome (DS) compared with another neurodevelopmental disorder, Williams syndrome (WS), and TD. Ten subjects with DS, 18 subjects with WS, and 40 subjects with TD each participated in a 3-Tesla MRI scan. We tested for group differences (DS vs. TD, DS vs. WS, and WS vs. TD) in between- and within-network rsFC connectivity for seven functional networks. For the DS group, we also examined associations between rsFC and other cognitive and genetic risk factors. In DS compared with TD, we observed higher levels of between-network connectivity in 6 out 21 network pairs but no differences in within-network connectivity. Participants with WS showed lower levels of within-network connectivity and no significant differences in between-network connectivity relative to DS. Finally, our comparison between WS and TD controls revealed lower within-network connectivity in multiple networks and higher between-network connectivity in one network pair relative to TD controls. While preliminary due to modest sample sizes, our findings suggest a global difference in between-network connectivity in individuals with neurodevelopmental disorders compared with controls and that such a difference is exacerbated across many brain regions in DS. However, this alteration in DS does not appear to extend to within-network connections, and therefore, the altered between-network connectivity must be interpreted within the framework of an intact intra-network pattern of activity. In contrast, WS shows markedly lower levels of within-network connectivity in the default mode network and somatomotor network relative to controls. These findings warrant further investigation using a task-based procedure that may help disentangle the relationship between brain function and cognitive performance across the spectrum of neurodevelopmental disorders.

"A Marriage on the Rocks": An Unknown Letter by William H. Kilpatrick about His Project Method

Science.gov (United States)

Knoll, Michael

2010-01-01

William H. Kilpatrick is worldwide known as "Mr. Project Method." But the origin of his celebrated paper of 1918 has never been explored. The discovery of a hitherto unknown letter reveals that Kilpatrick was an educational entrepreneur who, without regard for language and tradition, adopted the term "project" and used it in a provocative new way…

Music and Anxiety in Williams Syndrome: A Harmonious or Discordant Relationship?

Science.gov (United States)

Dykens, Elisabeth M.; Rosner, Beth A.; Ly, Tran; Sagun, Jaclyn

2005-01-01

In this two-part study, we assessed musical involvements in two samples of persons with Williams syndrome compared to others with mental retardation and also related musicality to anxiety and fears in Study 2. Relative to others with mental retardation, those with Williams syndrome were more likely to take music lessons, play an instrument, and…

Social phenotypes of autism spectrum disorders and Williams syndrome: similarities and differences

Directory of Open Access Journals (Sweden)

Kosuke eAsada

2012-07-01

Full Text Available Autism spectrum disorders (ASD and Williams syndrome (WS both are neurodevelopmental disorders, each with a unique social phenotypic pattern. This review article aims to define the similarities and differences between the social phenotypes of ASD and WS. We review studies that have examined individuals with WS using diagnostic assessments such as the Autism Diagnostic Observation Schedule (ADOS, cross-syndrome direct comparison studies, and studies that have individually examined either disorder. We conclude that (1 Individuals with these disorders show quite contrasting phenotypes for face processing (i.e., preference to faces and eyes and sociability (i.e., interest in and motivation to interact with others, and (2 Although the ADOS and a direct comparison study on pragmatic language ability suggest more deficits in ASD, individuals with WS are similarly impaired on social cognition and communicative skills. In light of these results, we discuss how cross-syndrome comparisons between ASD and WS can contribute to developmental theory, cognitive neuroscience, and the development and choice of clinical treatments.

Prince William Sound, Alaska ESI: HYDRO (Hydrology)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Prince William Sound, Alaska ESI: INVERT (Invertebrates)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Who Was the Real William Shakespeare?

Science.gov (United States)

Edwards, Michael Todd

2009-01-01

This article highlights a project that encourages students to connect reading and mathematics instruction by using a data analysis approach. Students analyze sonnets from statistical, literary, and historical points of view in an effort to uncover the true identity of William Shakespeare. (Contains 10 figures.)

Mr. William Shakespeare and the Internet.

Science.gov (United States)

Reeves, Barbara

2000-01-01

Describes resources and links on a Web site entitled "Mr. William Shakespeare and the Internet," with suggestions for using them with students. Highlights include: historical context; impact of events/situations on works; motivational/preparatory lessons; reading and understanding Shakespeare; analysis of works; language; assessing other…

[Vascular malformations in the Williams-Beuren syndrome: report of three new cases].

Science.gov (United States)

Sator, Hicham; Rhouni, Fatima Ezzahra; Benjouad, Ibitihale; Rhouni, Fatima Ezzahra; Benjouad, Ibitihale; Dafiri, Rachida; Chat, Latifa

2016-01-01

The Williams-Beuren syndrome is a rare genetic disease. It combines classically specific facial dysmorphism, cardiovascular malformations and specific neuropsychological profile. We report three cases of Williams-Beuren syndrome in children with particular emphasis on vascular abnormalities observed on CT angiography and MR angiography.

Energy policy options for Illinois. Proceedings. [26 papers

Energy Technology Data Exchange (ETDEWEB)

1977-01-01

Twenty-six papers presented at the Fifth Annual Oil Illinois Energy Conference are categorized into five sections, namely: An overview of U.S. and Illinois Energy Policy; Energy Policy; Conservation--Solar--Biomass and Solid Wastes; Energy Policy; Petroleum and Natural Gas; Energy Policy; Coal and Electric Utilities; and Economic and Consumer Concerns. One paper, A Perspective on Long-Range Nuclear Energy Options, by William O. Harms has previously appeared in EAPA 4: 1364. (MCW)

33 CFR 164.43 - Automatic Identification System Shipborne Equipment-Prince William Sound.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false Automatic Identification System Shipborne Equipment-Prince William Sound. 164.43 Section 164.43 Navigation and Navigable Waters COAST GUARD... Automatic Identification System Shipborne Equipment—Prince William Sound. (a) Until December 31, 2004, each...

77 FR 43276 - Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal...

Science.gov (United States)

2012-07-24

...; William D. Ford Federal Direct Loan Program General Forbearance Request SUMMARY: Borrowers who receive loans through the William D. Ford Federal Direct Loan Program will use this form to request forbearance... considered public records. Title of Collection: William D. Ford Federal Direct Loan Program General...

Seasonal distribution of Dall's porpoise in Prince William Sound, Alaska

Science.gov (United States)

Moran, John R.; O'Dell, Matthew B.; Arimitsu, Mayumi L.; Straley, Janice M.; Dickson, Danielle M. S.

2018-01-01

Dall's porpoise, Phocoenoides dalli, are a conspicuous predator in the Prince William Sound ecosystem, yet there has been little effort directed towards monitoring this species since the 1980s, prior to the Exxon Valdez oil spill. We used vessel-based surveys to examine the seasonal distribution of Dall's porpoise in the waters of Prince William Sound during eight years from 2007 to 2015. Over the course of 168 days and 15,653 km of survey effort, 921 Dall's porpoise were encountered in 210 groups. We estimate an encounter rate of 0.061 porpoise/km traveled or 1 porpoise encountered for every 16.5 km traveled. Dall's porpoise were found throughout the year in Prince William Sound, and used a wide range of habitats, including those not considered typical of the species, such as bays, shallow water, and nearshore waters. Dall's porpoise seasonally shifted their center of distribution from the western passages in fall to the bays of the eastern Sound in winter and spring. Dall's porpoises were widely dispersed throughout the Sound in summer. We identified potential Dall's porpoise habitat (depth, slope, and distance from shore) within Prince William Sound using generalized additive models (GAM). Dall's porpoise were found in deeper water during summer and in shallowest water during spring. We propose that their use of novel habitats is a function of reduced predation risk associated with the decline of their main predator, killer whales (Orcinus orca), following the Exxon Valdez oil spill, and the presence of overwintering and spawning Pacific herring (Clupea pallasii). While the size of the Dall's porpoise population within Prince William Sound remains unknown, our encounter rates were lower than those reported in the 1970s. Their high metabolic rate and ubiquitous presence makes them one of the more important, yet understudied, forage fish predators in the region.

Williams-Beuren syndrome associated with single kidney and nephrocalcinosis: a case report.

Science.gov (United States)

Abidi, Kamel; Jellouli, Manel; Ben Rabeh, Rania; Hammi, Yousra; Gargah, Tahar

2015-01-01

Williams-Beuren syndrome is a rare neurodevelopmental disorder, characterized by congenital heart defects, abnormal facial features, mental retardation with specific cognitive and behavioral profile, growth hormone deficiency, renal and skeletal anomalies, inguinal hernia, infantile hypercalcaemia. We report a case with Williams-Beuren syndrome associated with a single kidney and nephrocalcinosis complicated by hypercalcaemia. A male infant, aged 20 months presented growth retardation associated with a psychomotor impairment, dysmorphic features and nephrocalcinosis. He had also hypercalciuria and hypercalcemia. Echocardiography was normal. DMSA renal scintigraphy showed a single functioning kidney. The FISH generated one ELN signal in 20 metaphases read and found the presence of ELN deletion, with compatible Williams-Beuren syndrome.

77 FR 47374 - Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal...

Science.gov (United States)

2012-08-08

...; William D. Ford Federal Direct Loan (Direct Loan) Program/ Federal Family Loan (FFEL) Program: Deferment... which borrowers in the William D. Ford Federal Direct Loan (Direct Loan) and Federal Family Education... considered public records. Title of Collection: William D. Ford Federal Direct Loan (Direct Loan) Program...

Attention to Faces in Williams Syndrome

Science.gov (United States)

Riby, Deborah M.; Jones, Nicola; Brown, Philippa H.; Robinson, Lucy J.; Langton, Stephen R. H.; Bruce, Vicki; Riby, Leigh M.

2011-01-01

Williams syndrome (WS) is associated with distinct social behaviours. One component of the WS social phenotype is atypically prolonged face fixation. This behaviour co-exists with attention difficulties. Attention is multi-faceted and may impact on gaze behaviour in several ways. Four experiments assessed (i) attention capture by faces, (ii)…

'O' Rose Thou Art Sick': Floral Symbolism in William Blake's Poetry

Directory of Open Access Journals (Sweden)

Noelia Malla

2014-04-01

Full Text Available The primary aim of this paper is to analyse the symbolic implications of floral imagery in William Blake’s poetry. More specifically, this study explores the process of floral (resignification of William Blake’s Songs of Innocence (1789 and Songs of Experience (1794 as case studies. Since “Without contraries [there] is no progression” (Marriage of Heaven and Hell, plate 3, it can be argued that the Songs represent contrary aspects of the human condition that far from contradicting each other, establish a static contrast of shifting tensions and revaluation of the flower-image not only as a perfect symbol of the “vegetable” life rooted to the Earth but also as a figure longing to be free. In some sense at some level, the poetic-prophetic voice asserts in the Songs of Experience the state of corruption where man has fallen into. Ultimately, this study will explore how the failure to overcome the contrast that is suggested in the Songs will be deepened by the tragedy of Thel, which is symbolized by all unborn forces of life, all sterile seeds as an ultimate means of metaphorical regeneration throughout Poetry which constitutes in itself the Poet Prophet’s own means of transcending through art.

Enough room for Williams and IMF? / Paul Beckman

Index Scriptorium Estoniae

Beckman, Paul

1999-01-01

Lõppesid Leedu ja USA energeetikakompanii Williams International läbirääkimised Leedu naftakompleksis osaluse omandamise asjus. IMF uurib Leedu majanduslikku arengut, mida tehing Williamsiga komplitseerib

Whither Sir William?

Directory of Open Access Journals (Sweden)

Stephen J. Greenberg, MSLS, PhD

2017-04-01

Full Text Available There are times when something is simply so familiar that we can no longer see it at all. It can be a story, or a concept, or even a flesh-and-blood person. Familiarity breeds not only contempt, but a kind of invisibility as well. For too many of us, such is the case with Sir William Osler. In his time (1849–1919, many considered him to be one of the greatest practitioners, teachers, and writers ever in the field of medicine. He was instrumental in the founding of the Medical Library Association (MLA and was elected its second president.

ABORDAGEM DIAGNÓSTICA DA SÍNDROME DE WILLIAMS - BEUREN.

OpenAIRE

Santos, Marina Machado

2016-01-01

As anomalias cromossômicas causam fenótipos específicos e complexos resultantes de desequilíbrios na dose normal de genes localizados em um segmento cromossômico específico, sendo responsáveis por 60 % ou mais das síndromes genéticas identificáveis. A Síndrome de Williams-Beuren (SWB) caracteriza-se por anomalias múltiplas e deficiência intelectual, descrita independentemente por Williams et al. (1961) e por Beuren et al. (1962). A SWB consiste em uma doença genética causada pela microdeleção...

Florence Jessie Mac Williams (1917-1990)

Indian Academy of Sciences (India)

Home; Journals; Resonance – Journal of Science Education; Volume 10; Issue 1. Florence Jessie Mac Williams (1917-1990). Featured Scientist Volume 10 Issue 1 January 2005 pp 98-98. Fulltext. Click here to view fulltext PDF. Permanent link: https://www.ias.ac.in/article/fulltext/reso/010/01/0098-0098. Resonance ...

Metabolic abnormalities in Williams-Beuren syndrome.

Science.gov (United States)

Palacios-Verdú, María Gabriela; Segura-Puimedon, Maria; Borralleras, Cristina; Flores, Raquel; Del Campo, Miguel; Campuzano, Victoria; Pérez-Jurado, Luis Alberto

2015-04-01

Williams-Beuren syndrome (WBS, OMIM-194050) is a neurodevelopmental disorder with multisystemic manifestations caused by a 1.55-1.83 Mb deletion at 7q11.23 including 26-28 genes. Reported endocrine and metabolic abnormalities include transient hypercalcaemia of infancy, subclinical hypothyroidism in ∼ 30% of children and impaired glucose tolerance in ∼ 75% of adult individuals. The purpose of this study was to further study metabolic alterations in patients with WBS, as well as in several mouse models, to establish potential candidate genes. We analysed several metabolic parameters in a cohort of 154 individuals with WBS (data available from 69 to 151 cases per parameter), as well as in several mouse models with complete and partial deletions of the orthologous WBS locus, and searched for causative genes and potential modifiers. Triglyceride plasma levels were significantly decreased in individuals with WBS while cholesterol levels were slightly decreased compared with controls. Hyperbilirubinemia, mostly unconjugated, was found in 18.3% of WBS cases and correlated with subclinical hypothyroidism and hypotriglyceridemia, suggesting common pathogenic mechanisms. Haploinsufficiency at MLXIPL and increased penetrance for hypomorphic alleles at the UGT1A1 gene promoter might underlie the lipid and bilirubin alterations. Other disturbances included increased protein and iron levels, as well as the known subclinical hypothyroidism and glucose intolerance. Our results show that several unreported biochemical alterations, related to haploinsufficiency for specific genes at 7q11.23, are relatively common in WBS. The early diagnosis, follow-up and management of these metabolic disturbances could prevent long-term complications in this disorder. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Outcome in Adult Life for People with Williams Syndrome Results from a Survey of 239 Families

Science.gov (United States)

Howlin, P.; Udwin, O.

2006-01-01

BACKGROUND: Although there has been considerable research into the genotype and phenotype of Williams syndrome, there have been relatively few studies of long-term prognosis. As a preliminary to a more detailed investigation of adults with Williams syndrome, a parental questionnaire was distributed to members of the UK Williams Syndrome…

Coronary ostium occlusion by coronary cusp displacement in Williams syndrome.

Science.gov (United States)

Shiohama, Tadashi; Fujii, Katsunori; Ebata, Ryota; Funabashi, Nobusada; Matsumiya, Goro; Saito, Yuko Kazato; Takechi, Fumie; Yonemori, Yoko; Nakatani, Yukio; Shimojo, Naoki

2016-06-01

Williams syndrome is a contiguous gene deletion syndrome resulting from a heterozygous deletion on chromosome 7q11.23, and is characterized by distinctive facial features and supravalvular aortic stenosis (SVAS). This syndrome rarely presents unpredictable cardiac death, and yet, as illustrated in the present case, it is still not possible to predict it, even on close monitoring. We herein describe the case of a 6-year-old Japanese girl with Williams syndrome, who had sudden cardiac collapse due to cardiac infarction after pharyngitis. Cardiac failure followed a critical course that did not respond to catecholamine support or heart rest with extracardiac mechanical support. Although marked coronary stenosis was not present, the left coronary cusp abnormally adhered to the aortic wall, which may synergistically cause coronary ostium occlusion with SVAS. Altered hemodynamic state, even that caused by the common cold, may lead to critical myocardial events in Williams syndrome with SVAS. © 2015 Japan Pediatric Society.

Who Invented the Word Asteroid: William Herschel or Stephen Weston?

Science.gov (United States)

Cunningham, Clifford J.; Orchiston, Wayne

2011-11-01

William Herschel made the first serious study of 1 Ceres and 2 Pallas in the year 1802. He was moved by their dissimilarities to the other planets to coin a new term to distinguish them. For this purpose he enlisted the aid of his good friends William Watson and Sir Joseph Banks. Watson gave him a long list of possible names, which Herschel rejected. With a lifetime of experience classifying and naming newly found objects in nature, Banks became the man both Erasmus Darwin (in 1781) and William Herschel (in 1802) turned to for sage advice in developing a new descriptive language. In the case of Ceres and Pallas, Banks turned the task over to his friend, the noted philologist Stephen Weston, FRS. It has recently been stated by a noted British historian that it was Weston - not Herschel - who coined the term 'asteroid' to collectively describe Ceres and Pallas. This claim is investigated, and parallels are drawn in the use of neologism in astronomy and botany.

Facilitating complex shape drawing in Williams syndrome and typical development.

Science.gov (United States)

Hudson, Kerry D; Farran, Emily K

2013-07-01

Individuals with Williams syndrome (WS) produce drawings that are disorganised, likely due to an inability to replicate numerous spatial relations between parts. This study attempted to circumvent these drawing deficits in WS when copying complex combinations of one, two and three shapes. Drawing decisions were reduced by introducing a number of facilitators, for example, by using distinct colours and including facilitatory cues on the response sheet. Overall, facilitation improved drawing in the WS group to a comparable level of accuracy as typically developing participants (matched for non-verbal ability). Drawing accuracy was greatest in both groups when planning demands (e.g. starting location, line lengths and changes in direction) were reduced by use of coloured figures and providing easily distinguished and clearly grouped facilitatory cues to form each shape. This study provides the first encouraging evidence to suggest that drawing of complex shapes in WS can be facilitated; individuals with WS might be receptive to remediation programmes for drawing and handwriting. Copyright © 2013 Elsevier Ltd. All rights reserved.

The scientific legacy of William Herschel

CERN Document Server

2018-01-01

This book presents a modern scholarly analysis of issues associated with England’s most famous astronomer, William Herschel. The world’s leading experts on Herschel, discoverer of the planet Uranus, here offer their combined wisdom on many aspects of his life and astronomical research. Solar system topics include comets, Earth’s Moon, and the spurious moons of Uranus, all objects whose observation was pioneered by Herschel.  The contributors examine his study of the structure of the Milky Way and an in-depth look at the development of the front view telescopes he built. The popular subject of extraterrestrial life is looked at from the point of view of both William Herschel and his son John, both of whom had an interest in the topic. William’s personal development through the educational system of the late eighteenth-century is also explored, and the wide range of verse and satire in various languages associated with his discoveries is collected here for the first time. Hershel worked at a time of i...

Post-Mao China: Educational Services for Exceptional Individuals

Science.gov (United States)

Ding, Yi; Yang, Ling-Yan; Xiao, Fei; Van Dyke, Don C.

2008-01-01

When William Moore, a Scottish Presbyterian pastor, established the first special school in China in 1874, the country began her long and circuitous journey toward establishing formal educational services for individuals with special needs. Special education in China developed slowly on the infertile soil of continual wars, political instability,…

Holocene deposition and megathrust splay fault geometries within Prince William Sound, Alaska

Science.gov (United States)

Finn, S.; Liberty, L. M.; Haeussler, P. J.; Pratt, T. L.

2011-12-01

New high resolution sparker seismic reflection data, in conjunction with reprocessed legacy seismic data, provide the basis for a new fault, fold, and Holocene sediment thickness database for Prince William Sound, Alaska. Additionally, legacy airgun seismic data in Prince William Sound and the Gulf of Alaska tie features on these new sparker data to deeper portions of megathrust splay faults. We correlate regionally extensive bathymetric lineaments within Prince William Sound to megathrust splay faults, such as the ones that ruptured in the 1964 M9.2 earthquake. Lastly, we estimate Holocene sediment thickness within Prince William Sound to better constrain the Holocene fault history throughout the region. We identify three seismic facies related to Holocene, Quaternary, and Tertiary strata that are crosscut by numerous high angle normal faults in the hanging wall of the megathrust splay faults. The crustal-scale seismic reflection profiles show splay faults emerging from 20 km depth between the Yakutat block and North American crust and surfacing as the Hanning Bay and Patton Bay faults. A change in exhumation rates, slip rates, and fault orientation appears near Hinchinbrook that we attribute to differences in subducted slab geometry. Based on our slip rate analysis, we calculate average Holocene displacements of 20 m and 100 m in eastern and western Prince William Sound, respectively. Landward of two splay faults exposed on Montague Island, we observe subsidence, faulting, and landslides that record deformation associated with the 1964 and older megathrust earthquakes.

March 1964 Prince William Sound, USA Images

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — The Prince William Sound magnitude 9.2 Mw earthquake on March 28, 1964 at 03:36 GMT (March 27 at 5:36 pm local time), was the largest U.S. earthquake ever recorded...

Attentional Disengagement in Adults with Williams Syndrome

Science.gov (United States)

Lense, Miriam D.; Key, Alexandra P.; Dykens, Elisabeth M.

2011-01-01

Williams syndrome (WS) is a neurodevelopmental disorder characterized by a distinctive behavioral and cognitive profile, including widespread problems with attention. However, the specific nature of their attentional difficulties, such as inappropriate attentional allocation and/or poor attentional disengagement abilities, has yet to be…

Robert Williams Wood: pioneer of invisible light.

Science.gov (United States)

Sharma, Shruti; Sharma, Amit

2016-03-01

The Wood's lamp aids in the diagnosis of multiple infectious, inflammatory and neoplastic dermatologic conditions. Although the Wood's lamp has many applications, which have improved both the diagnosis and management of disease, the man credited for its invention is relatively unknown in medicine. Robert Williams Wood, a prominent physicist of the early 20th century, is credited for the invention of the Wood's lamp. Wood was the father of infrared and ultraviolet photography and made significant contributions to other areas in optics and spectroscopy. Wood's work encompassed the formative years of American Physics; he published over 200 original papers over his lifetime. A few years after the invention of the Wood's lamp for ultraviolet photography, physicians in Europe adopted the Wood's lamp for dermatologic applications. Wood's lamp remains popular in clinics globally, given its ease of use and ability to improve diagnostic precision. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Sleep-EEG in dizygotic twins discordant for Williams syndrome.

Science.gov (United States)

Bódizs, Róbert; Gombos, Ferenc; Szocs, Katalin; Réthelyi, János M; Gerván, Patrícia; Kovács, Ilona

2014-01-30

Reports on twin pairs concordant and discordant for Williams syndrome were published before, but no study unravelled sleep physiology in these cases yet. We aim to fill this gap by analyzing sleep records of a twin pair discordant for Williams syndrome extending our focus on presleep wakefulness and sleep spindling. We performed multiplex ligation-dependent probe amplification of the 7q11.23 region of a 17 years old dizygotic opposite-sex twin pair discordant for Williams syndrome. Polysomnography of laboratory sleep at this age was analyzed and followed-up after 1.5 years by ambulatory polysomnography. Sleep stages scoring, EEG power spectra and sleep spindle analyses were carried out. The twin brother showed reduced levels of amplification for all of the probes in the 7q11.23 region indicating a typical deletion spanning at least 1.038 Mb between FKBP6 and CLIP2. The results of the twin sister showed normal copy numbers in the investigated region. Lower sleep times and efficiencies, as well as higher slow wave sleep percents of the twin brother were evident during both recordings. Roughly equal NREM, Stage 2 and REM sleep percents were found. EEG analyses revealed state and derivation-independent decreases in alpha power, lack of an alpha spectral peak in presleep wakefulness, as well as higher NREM sleep sigma peak frequency in the twin brother. Faster sleep spindles with lower amplitude and shorter duration characterized the records of the twin brother. Spectra show a striking reliability and correspondence between the two situations (laboratory vs. home records). Alterations in sleep and specific neural oscillations including the alpha/sigma waves are inherent aspects of Williams syndrome.

Variations of transcript profiles between sea otters Enhydra lutris from Prince William Sound, Alaska, and clinically normal reference otters

Science.gov (United States)

Miles, A. Keith; Bowen, Lizabeth; Ballachey, Brenda E.; Bodkin, James L.; Murray, M.; Estes, J.L.; Keister, Robin A.; Stott, J.L.

2012-01-01

Development of blood leukocyte gene transcript profiles has the potential to expand condition assessments beyond those currently available to evaluate wildlife health, including sea otters Enhydra lutris, both individually and as populations. The 10 genes targeted in our study represent multiple physiological systems that play a role in immuno-modulation, inflammation, cell protection, tumor suppression, cellular stress-response, xenobiotic metabolizing enzymes, and antioxidant enzymes. These genes can be modified by biological, physical, or anthropogenic impacts and consequently provide information on the general type of stressors present in a given environment. We compared gene transcript profiles of sea otters sampled in 2008 among areas within Prince William Sound impacted to varying degrees by the 1989 ‘Exxon Valdez’ oil spill with those of captive and wild reference sea otters. Profiles of sea otters from Prince William Sound showed elevated transcription in genes associated with tumor formation, cell death, organic exposure, inflammation, and viral exposure when compared to the reference sea otter group, indicating possible recent and chronic exposure to organic contaminants. Sea otters from historically designated oiled areas within Prince William Sound 19 yr after the oil spill had higher transcription of genes associated with tumor formation, cell death, heat shock, and inflammation than those from areas designated as less impacted by the spill.

Auditory Attraction: Activation of Visual Cortex by Music and Sound in Williams Syndrome

Science.gov (United States)

Thornton-Wells, Tricia A.; Cannistraci, Christopher J.; Anderson, Adam W.; Kim, Chai-Youn; Eapen, Mariam; Gore, John C.; Blake, Randolph; Dykens, Elisabeth M.

2010-01-01

Williams syndrome is a genetic neurodevelopmental disorder with a distinctive phenotype, including cognitive-linguistic features, nonsocial anxiety, and a strong attraction to music. We performed functional MRI studies examining brain responses to musical and other types of auditory stimuli in young adults with Williams syndrome and typically…

William James, Gustav Fechner, and Early Psychophysics

Directory of Open Access Journals (Sweden)

Stephanie L. Hawkins

2011-10-01

Full Text Available American psychologist and philosopher William James devoted the entirety of his career to exploring the nature of volition, as expressed by such phenomena as will, attention, and belief. As part of that endeavor, James's unorthodox scientific pursuits, from his experiments with nitrous oxide and hallucinogenic drugs to his investigation of spiritualist mediums, represent his attempt to address the "hard problems" of consciousness for which his training in brain physiology and experimental psychology could not entirely account. As a student, James's reading in chemistry and physics had sparked his interest in the concepts of energy and force, terms that he later deployed in his writing about consciousness and in his arguments against philosophical monism and scientific materialism, as he developed his radically empiricist ideas privileging discontinuity and plurality. Despite James's long campaign against scientific materialism, he was, however, convinced of the existence of a naturalistic explanation for the more "wayward and fitful" aspects of mind, including transcendent experiences associated with hysteria, genius, and religious ecstasy. In this paper, I examine aspects of James's thought that are still important for contemporary debates in psychology and neuroscience: his "transmission theory" of consciousness, his ideas on the "knowing of things together," and, finally, the related concept of "the compounding of consciousness," which postulates the theoretical possibility for individual entities within a conscious system of thought to "know" the thoughts of others within the system. Taken together, these ideas suggest that James, in spite of, or perhaps because of, his forays into metaphysics, was working toward a naturalistic understanding of consciousness, what I will term a "distributive model," based on his understanding of consciousness as an "awareness" that interacts dynamically within, and in relation to, its environment.

Florence Jessie MacWilliams (1917-1990)

Indian Academy of Sciences (India)

famous mathematician Oscar Zariski, well known for his work in algebraic geometry, at. Johns Hopkins University, following him to Harvard University to study with him for a year. There was a break in her studies for many years following her marriage in 1941 to. W al ter Mac Williams, an engineer, and the birth and raising of ...

Prince William Sound, Alaska ESI: FISH (Fish Polygons)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Prince William Sound, Alaska ESI: NESTS (Bird Nests)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

De wereld in 18 lessen: Christopher Williams

NARCIS (Netherlands)

Berrebi, S.

2011-01-01

Hij is een echte artist’s artist, de conceptuele fotograaf Christopher Williams. Sinds hij in Duitsland woont en werkt, vindt zijn afgewogen werk, dat de fotografie tegelijk eert en ondervraagt, steeds makkelijker onderdak in Europese kunstinstituten, zoals dit voorjaar bij Museum Dhondt-Dhaenens in

Dissociation of spatial memory systems in Williams syndrome.

Science.gov (United States)

Bostelmann, Mathilde; Fragnière, Emilie; Costanzo, Floriana; Di Vara, Silvia; Menghini, Deny; Vicari, Stefano; Lavenex, Pierre; Lavenex, Pamela Banta

2017-11-01

Williams syndrome (WS), a genetic deletion syndrome, is characterized by severe visuospatial deficits affecting performance on both tabletop spatial tasks and on tasks which assess orientation and navigation. Nevertheless, previous studies of WS spatial capacities have ignored the fact that two different spatial memory systems are believed to contribute parallel spatial representations supporting navigation. The place learning system depends on the hippocampal formation and creates flexible relational representations of the environment, also known as cognitive maps. The spatial response learning system depends on the striatum and creates fixed stimulus-response representations, also known as habits. Indeed, no study assessing WS spatial competence has used tasks which selectively target these two spatial memory systems. Here, we report that individuals with WS exhibit a dissociation in their spatial abilities subserved by these two memory systems. As compared to typically developing (TD) children in the same mental age range, place learning performance was impaired in individuals with WS. In contrast, their spatial response learning performance was facilitated. Our findings in individuals with WS and TD children suggest that place learning and response learning interact competitively to control the behavioral strategies normally used to support human spatial navigation. Our findings further suggest that the neural pathways supporting place learning may be affected by the genetic deletion that characterizes WS, whereas those supporting response learning may be relatively preserved. The dissociation observed between these two spatial memory systems provides a coherent theoretical framework to characterize the spatial abilities of individuals with WS, and may lead to the development of new learning strategies based on their facilitated response learning abilities. © 2017 Wiley Periodicals, Inc.

A case of William's syndrome associated peripheral pulmonary arterial stenosis

Energy Technology Data Exchange (ETDEWEB)

Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan [College of Medicine, Yeungam University, Daegu (Korea, Republic of)

1988-06-15

William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures.

Word-Preserving Arts: Material Inscription, Ekphrasis, and Spatial Form in the Later Work of William Wordsworth

DEFF Research Database (Denmark)

Simonsen, Peter

William Wordsworth, posterity, reading, material inscription, ekphrasis, description, sonnet, portraiture......William Wordsworth, posterity, reading, material inscription, ekphrasis, description, sonnet, portraiture...

Writing abilities in intellectual disabilities: a comparison between Down and Williams syndrome.

Science.gov (United States)

Varuzza, Cristiana; De Rose, Paola; Vicari, Stefano; Menghini, Deny

2015-02-01

Writing is a complex task that requires the integration of multiple cognitive, linguistic, and motor abilities. Until now, only a few studies investigated writing abilities in individuals with Intellectual Disability (ID). The aim of the present exploratory study was to provide knowledge on the organization of writing in two populations with ID, Down syndrome (DS) and Williams syndrome (WS), trying to disentangle different components of the process. A battery tapping diverse writing demands as low-level transcription skills as well as high-level writing skills was proposed to 13 individuals with WS, 12 individuals with DS and 11 mental-age-matched typically developing (TD) children. Results showed that the two groups with genetic syndromes did not differ from TD in writing a list of objects placed in bedroom, in the number of errors in the text composition, in a text copying task and in kind of errors made. However, in a word dictation task, individuals with DS made more errors than individuals with WS and TD children. In a pseudoword dictation task, both individuals with DS and WS showed more errors than TD children. Our results showed good abilities in individuals with ID in different aspects of writing, involving not only low-level transcription skills but also high-level composition skills. Contrary to the pessimistic view, considering individuals with ID vulnerable for failure, our results indicate that the presence of ID does not prevent the achievement of writing skills. Copyright © 2014 Elsevier Ltd. All rights reserved.

Minor Characters in William Shakespeare's Twelfth Night and A Midsummer Night's Dream

Directory of Open Access Journals (Sweden)

Zahraa Adnan Baqer

2018-01-01

Full Text Available This paper aims at discussing the role of the minor characters in William Shakespeare's Twelfth Night and A Midsummer Night's Dream. The study assumes that without the first group of minor characters, associated with Olivia, the play Twelfth Night would lose much of its humor, and without the second group, associated with Sebastian, the play would fall apart. On the other hand, in Shakespeare's A Midsummer Night's Dream minor characters play important roles, without them, the action dose not ran smoothly, or does not ran at all. The paper falls into three sections. Section one deals with the role of each minor character in Twelfth Night.  Section two focuses on the minor characters in A Midsummer Night's Dream. Section three is a conclusion which sums up the findings of the study.

The world in eighteen lessons: Christopher Williams

NARCIS (Netherlands)

Berrebi, S.

2011-01-01

Conceptual photographer Christopher Williams is a real artist's artist. Ever since he moved to Germany, his measured work, which both reveres and examines the art of photography, has more and more easily found its way into European art institutes, such as this spring at Museum Dhondt-Dhaenens in

Analysis of Speech Fluency in Williams Syndrome

Science.gov (United States)

Rossi, Natalia Freitas; Sampaio, Adriana; Goncalves, Oscar F.; Giacheti, Celia Maria

2011-01-01

Williams syndrome (WS) is a neurodevelopmental genetic disorder, often referred as being characterized by dissociation between verbal and non-verbal abilities, although the number of studies disputing this proposal is emerging. Indeed, although they have been traditionally reported as displaying increased speech fluency, this topic has not been…

Attribution of Negative Intention in Williams Syndrome

Science.gov (United States)

Godbee, Kali; Porter, Melanie A.

2013-01-01

People with Williams syndrome (WS) are said to have sociable and extremely trusting personalities, approaching strangers without hesitation. This study investigated whether people with WS are less likely than controls to attribute negative intent to others when interpreting a series of ambiguous pictures. This may, at least partially, explain…

William Barlow and the Determination of Atomic Arrangement in Crystals.

Science.gov (United States)

Mauskopf, Seymour H

2015-04-01

William Barlow (1845-1934) was an important if unconventional scientist, known for having developed the 'closest-packing' atomic models of crystal structure. He resumed an early nineteenth-century tradition of utilizing crystallographical and chemical data to determine atomic arrangements in crystals. This essay recounts Barlow's career and scientific activity in three parts: (a) His place in the tradition of determining atomic arrangement in context of this earlier tradition and of contemporaneous developments of crystallography and chemistry, (b) his unconventional career, and (c) the 'success' of his program to determine atomic arrangements in crystals and its influence on the work of William Lawrence Bragg.

An Investigation into Japine Platinum Photographs: William Willis´s Proprietary Paper

DEFF Research Database (Denmark)

Vila, Anna; Clark, Matthew L; McCabe, Constance

2015-01-01

and are essentially unknown to modern-day scholars who may mistake them as prints with thin binder layers, such as albumen or gelatin. Indeed, the approach to conservation of these photographs may differ from one designed for a traditional platinum print. The parchmentized Japine paper was criticized for its tendency......Analyses of two samples of the commercial platinum photographic paper “Japine” revealed that this trade-name product was sold with a range of surface properties from matte to glossy. An in-depth examination of an unprocessed glossy Japine sample showed a chemically modified surface resulting from...... parchmentization by treatment with sulfuric acid. This treatment results in a thin film of amorphous cellulose on the printing surface, altering its visual appearance and wet strength. Photographers selected this paper due to its aesthetic qualities. However, such papers have never been characterized...

Ocular Features in 16 Brazilian Patients with Williams-Beuren Syndrome.

Science.gov (United States)

Viana, Melissa Machado; Frasson, Maria; Galvão, Henrique; Leão, Letícia Lima; Stofanko, Martin; Gonçalves-Dornelas, Higgor; da Silva Cunha, Pricila; Burle de Aguiar, Marcos José

2015-01-01

Williams-Beuren Syndrome (WBS) is a multisystem disorder caused by the deletion of contiguous genes on chromosome 7q11.23. Ophthalmologic abnormalities and deficits in visual motor integration are important features of WBS. Here we describe our experience with Brazilian WBS patients and their ophthalmologic features. Sixteen patients with confirmed WBS went through thorough ophthalmologic examination. The most frequent ocular findings in our group of patients were stellate iris pattern (81.2%), hyperopic astigmatism (50%), hyperopia (37.5%), tortuosity of retinal vessel (37.5%) and strabismus (18.7%). This is the second report of ophthalmologic abnormalities in a group of Brazilian individuals with WBS. It is extremely valuable that specific populations are studied so that clinical diagnosis can be refined and management of patients can be driven to the most common presentations of the disease.

77 FR 63308 - J. William Foley Incorporated v. United Illuminating Company; Notice of Complaint

Science.gov (United States)

2012-10-16

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. EL12-106-000] J. William Foley Incorporated v. United Illuminating Company; Notice of Complaint Take notice that on October 5...) and 825(e), J. William Foley Incorporated (Complainant) filed a formal complaint against United...

Application of Choi—Williams Reduced Interference Time Frequency Distribution to Machinery Diagnostics

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Howard A. Gaberson

1995-01-01

Full Text Available This article discusses time frequency analysis of machinery diagnostic vibration signals. The short time Fourier transform, the Wigner, and the Choi–Williams distributions are explained and illustrated with test cases. Examples of Choi—Williams analyses of machinery vibration signals are presented. The analyses detect discontinuities in the signals and their timing, amplitude and frequency modulation, and the presence of different components in a vibration signal.

Increased overall cortical connectivity with syndrome specific local decreases suggested by atypical sleep-EEG synchronization in Williams syndrome.

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Gombos, Ferenc; Bódizs, Róbert; Kovács, Ilona

2017-07-21

Williams syndrome (7q11.23 microdeletion) is characterized by specific alterations in neurocognitive architecture and functioning, as well as disordered sleep. Here we analyze the region, sleep state and frequency-specific EEG synchronization of whole night sleep recordings of 21 Williams syndrome and 21 typically developing age- and gender-matched subjects by calculating weighted phase lag indexes. We found broadband increases in inter- and intrahemispheric neural connectivity for both NREM and REM sleep EEG of Williams syndrome subjects. These effects consisted of increased theta, high sigma, and beta/low gamma synchronization, whereas alpha synchronization was characterized by a peculiar Williams syndrome-specific decrease during NREM states (intra- and interhemispheric centro-temporal) and REM phases of sleep (occipital intra-area synchronization). We also found a decrease in short range, occipital connectivity of NREM sleep EEG theta activity. The striking increased overall synchronization of sleep EEG in Williams syndrome subjects is consistent with the recently reported increase in synaptic and dendritic density in stem-cell based Williams syndrome models, whereas decreased alpha and occipital connectivity might reflect and underpin the altered microarchitecture of primary visual cortex and disordered visuospatial functioning of Williams syndrome subjects.

Notas no percurso: linguagem musical e Síndrome de Williams/Notes on the way: musical language and Williams Syndrome

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Paulo Sérgio Souza Junior

2009-12-01

Full Text Available O presente artigo versa sobre uma síndrome em particular chamada Síndrome de Williams-Beuren. Esta síndrome possui uma relação específica com a música, relação esta que estará no fulcro de nossas preocupações durante as páginas que se seguem. Num primeiro momento faremos uma apresentação da síndrome e de suas singularidades; em seguida nos debruçaremos sobre a especificidade que aqui está em questão, ou seja, a relação da síndrome com o universo sonoro; por fim, traremos um caso com o qual tivemos contato envolvendo a educação musical em contexto da síndrome, e discutiremos a respeito dos elementos que esse contato nos trouxe para pensar essa curiosa e relevante relação entre arte-educação e o desenvolvimento neuropsicológico atípico. The present article is about a particular syndrome, called Williams-Beuren. This syndrome has a very specific relationship with music, which will be at the heart of our concerns over the following pages. Initially we will make a presentation of the syndrome and its singularities. Then we will focus on the uniqueness of the correlation between Williams-Beuren and the musical world. Finally, we will discuss a case which involves musical education in the context of this syndrome, and discuss about the elements, brought by that contact, that are useful to think about this curious and relevant relationship between art education and atypical neuropsychological development.

77 FR 58819 - Notice of Submission for OMB Review; Federal Student Aid; William D. Ford Federal Direct Loan...

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2012-09-24

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2012-05-21

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Shepard Award Winners, Part 2: Dr. Tracie Williams

Centers for Disease Control (CDC) Podcasts

2009-07-29

This podcast highlights the accomplishments of Dr. Tracie Williams, recipient of the prestigious 2009 CDC Charles C. Shepard Award.  Created: 7/29/2009 by Centers for Disease Control and Prevention (CDC).   Date Released: 7/29/2009.

Sir William Jenner (1815-1898) and Queen Victoria.

Science.gov (United States)

Seddon, David; Queen Victoria

2004-08-01

This biographical note outlines the work of Sir William Jenner (1815-1898), court physician 1861-93 and President of the Royal College of Physicians 1881-8. He was the first to distinguish between typhoid and typhus both clinically and pathologically.

The contribution of CLIP2 haploinsufficiency to the clinical manifestations of the Williams-Beuren syndrome.

Science.gov (United States)

Vandeweyer, Geert; Van der Aa, Nathalie; Reyniers, Edwin; Kooy, R Frank

2012-06-08

Williams-Beuren syndrome is a rare contiguous gene syndrome, characterized by intellectual disability, facial dysmorphisms, connective-tissue abnormalities, cardiac defects, structural brain abnormalities, and transient infantile hypercalcemia. Genes lying telomeric to RFC2, including CLIP2, GTF2I and GTF2IRD1, are currently thought to be the most likely major contributors to the typical Williams syndrome cognitive profile, characterized by a better-than-expected auditory rote-memory ability, a relative sparing of language capabilities, and a severe visual-spatial constructive impairment. Atypical deletions in the region have helped to establish genotype-phenotype correlations. So far, however, hardly any deletions affecting only a single gene in the disease region have been described. We present here two healthy siblings with a pure, hemizygous deletion of CLIP2. A putative role in the cognitive and behavioral abnormalities seen in Williams-Beuren patients has been suggested for this gene on the basis of observations in a knock-out mouse model. The presented siblings did not show any of the clinical features associated with the syndrome. Cognitive testing showed an average IQ for both and no indication of the Williams syndrome cognitive profile. This shows that CLIP2 haploinsufficiency by itself does not lead to the physical or cognitive characteristics of the Williams-Beuren syndrome, nor does it lead to the Williams syndrome cognitive profile. Although contribution of CLIP2 to the phenotype cannot be excluded when it is deleted in combination with other genes, our results support the hypothesis that GTF2IRD1 and GTF2I are the main genes causing the cognitive defects associated with Williams-Beuren syndrome. Copyright © 2012 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

Brief Report: Developing Spatial Frequency Biases for Face Recognition in Autism and Williams Syndrome

Science.gov (United States)

Leonard, Hayley C.; Annaz, Dagmara; Karmiloff-Smith, Annette; Johnson, Mark H.

2011-01-01

The current study investigated whether contrasting face recognition abilities in autism and Williams syndrome could be explained by different spatial frequency biases over developmental time. Typically-developing children and groups with Williams syndrome and autism were asked to recognise faces in which low, middle and high spatial frequency…

Denigrating Carl Rogers: William Coulson's Last Crusade.

Science.gov (United States)

Kirschenbaum, Howard

1991-01-01

Reviews William Coulson's assertions that Carl Rogers, Abraham Maslow, and he initiated the humanistic education field, that Rogers repudiated his philosophy late in life, and that they owe the nation's parents an apology. Argues that these charges are groundless and provides examples and quotations from Rogers' later writings to show how Rogers…

William, a voxel model of child anatomy from tomographic images for Monte Carlo dosimetry calculations

International Nuclear Information System (INIS)

Caon, M.

2010-01-01

Full text: Medical imaging provides two-dimensional pictures of the human internal anatomy from which may be constructed a three-dimensional model of organs and tissues suitable for calculation of dose from radiation. Diagnostic CT provides the greatest exposure to radiation per examination and the frequency of CT examination is high. Esti mates of dose from diagnostic radiography are still determined from data derived from geometric models (rather than anatomical models), models scaled from adult bodies (rather than bodies of children) and CT scanner hardware that is no longer used. The aim of anatomical modelling is to produce a mathematical representation of internal anatomy that has organs of realistic size, shape and positioning. The organs and tissues are represented by a great many cuboidal volumes (voxels). The conversion of medical images to voxels is called segmentation and on completion every pixel in an image is assigned to a tissue or organ. Segmentation is time consuming. An image processing pack age is used to identify organ boundaries in each image. Thirty to forty tomographic voxel models of anatomy have been reported in the literature. Each model is of an individual, or a composite from several individuals. Images of children are particularly scarce. So there remains a need for more paediatric anatomical models. I am working on segmenting ''William'' who is 368 PET-CT images from head to toe of a seven year old boy. William will be used for Monte Carlo dose calculations of dose from CT examination using a simulated modern CT scanner.

Revolution and Exploration: the English Translations of Rousseau and Humboldt by Helen Maria Williams

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Paula Yurss Lasanta

2017-12-01

Full Text Available British author Helen Maria Williams (1759-1827 was a well-known figure in the eighteenth century literary circles, whose work was praised by Elizabeth Montagu, Samuel Johnson, Dorothy and William Wordsworth, Mary Wollstonecraft, Hester Piozzi or Alexander von Humboldt.  In her early poems  Edwin and Eltruda (1782, An Ode to the Peace (1783 and Peru (1784, Williams starts to reveal her political tendencies by appealing to strong empathic feelings as a key to social and political transformation. As a result of her interest in politics, she travelled to France in 1790 and published her most acclaimed work Letters from France (1790. However, the rest of her production has received little critical attention by modern scholars, who have overlooked her involvement in translation. Williams’ only extant novel, Julia (1790 is in fact a creative translation of Rousseau’s Julie ou La Nouvelle Héloïse (1761, in which Williams includes poems that evidence her interest in revolutionary politics. Four years later, she translated Bernardin de Saint Pierre’s Paul et Virginie, while she was imprisoned in Paris. While translating novels was regarded as a respectable exercise for women writers, Williams challenges gender assumptions by translating Researches (1814 and the seven volumes of Personal Narrative (1814-1829, which had been produced by one of the most influential eighteenth century scientists, Alexander von Humboldt. This article interrogates how Williams makes use of translation to access areas of knowledge traditionally restricted to men, such as philosophy, politics and science. For this purpose, I will focus on her translations of the work of two leading intellectual figures of the eighteenth century,  Rousseau and Von Humboldt.

Läbi legendide William Shakespeare'i poole / Maris Peters

Index Scriptorium Estoniae

Peters, Maris

2010-01-01

Tutvustus: Wells, Stanley. Kas on tõsi, et Shakespeare ...? / tõlkinud Maris Peters. Tallinn : Argo, 2010. Raamat William Shakespeare kohta käivatest legendidest, kuuldustest ja teooriatest ning tema teoste autorsusest

On the application of the Williams-Weizsaecker-method to higher order S-matrix-approximations

International Nuclear Information System (INIS)

Ziegelbecker, R.C.

1983-05-01

In this paper the method of quasireal processes is investigated using a special example - pair production in the stationary field of a nucleus by an incident electron. As a result, the semi-classical version of the Williams-Weizsaecker-method is confirmed on the basis of all 3sup(rd)-order Feynman-diagrams. The spectra of quasireal processes, derived from quantum field theory, can also be applied simultaneously in several vertex points on one diagram and are valid for higher photon energies than the semiclassical spectrum; the restriction #betta# [de

William and Caroline Herschel pioneers in late 18th-century astronomy

CERN Document Server

Hoskin, Michael

2013-01-01

This beautifully structured book presents the essentials of William and Caroline Herschel's pioneering achievements in late 18th-century astronomy. Michael Hoskin shows that William Herschel was the first observational cosmologist and one of the first observers to attack the sidereal universe beyond the solar system:Herschel built instruments far better than any being used at the royal observatory. Aided by his sister Caroline, he commenced a great systematic survey that led to his discovery of Uranus in 1781.Unlike observers before him, whose telescopes did not reveal them as astronomical obj

In Search of William Gascoigne Seventeenth Century Astronomer

CERN Document Server

Sellers, David

2012-01-01

William Gascoigne (c.1612-44), the first inventor of the telescopic sight and micrometer—instruments crucial to the advance of astronomy—was killed in the English Civil War. His name is now known to historians of science around the world, but for some considerable time after his tragic death at the age of 32, it seemed as if his achievements would be consigned to oblivion. Most of his papers were lost in the maelstrom of war and the few that seemed to have survived later disappeared. This is the story of how his work was rescued. Woven into that story is an account of the state of astronomy and optics during Gascoigne’s lifetime, so that the reader can appreciate the significance of his discoveries.   A substantial appendix presents selected extracts from Gascoigne's correspondence. This includes much new material that has not been previously published and illustrates his efforts to explain the basis of his pioneering techniques to the satisfaction of his contemporaries.   

Herbert Hoover and William Shakespeare: Two Writers on Ethics.

Science.gov (United States)

Somers, Margaret L.

1979-01-01

Outlines the ways in which Herbert Hoover and William Shakespeare wrote about professional ethics (for engineers and kings, respectively) using the writing techniques of concreteness, audience awareness, and development by induction. (TJ)

Sir William Turner (1832-1916) - Lancastrian, anatomist and champion of the Victorian era.

Science.gov (United States)

Wessels, Quenton; Correia, Janine Carla; Taylor, Adam M

2016-11-01

Sir William Turner, a Lancastrian, was renowned as a scientist, anatomist and a great reformer of medical education. His students became anatomists at various international institutions, which consequently shaped the future of anatomy as a subject matter both in the United Kingdom and in South Africa. Although Turner's accomplishments have been documented, little is known about the details that determined his career path and the individuals that shaped his future. Here the authors aim to highlight some aspects of Turner's academic achievements and his personal life as well as how he crossed paths with other great minds of the Victorian era including Richard Owen, Charles Darwin, James Paget and Joseph Lister. © The Author(s) 2015.

William Franke, Sulla verità poetica che è superiore alla Storia: Porfirio e la critica filosofica della letteratura William Franke, On the Poetic Truth that is Higher than History: Porphyry and the Philosophical Interpretation of Literature

Directory of Open Access Journals (Sweden)

Laura Lucia Rossi

2010-06-01

Full Text Available

78 FR 45515 - Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan...

Science.gov (United States)

2013-07-29

... DEPARTMENT OF EDUCATION [Docket No.: ED-2013-ICCD-0099] Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan Program Repayment Plan Selection Form AGENCY... notice will be considered public records. Title of Collection: William D. Ford Federal Direct Loan...

Psycholinguistic Abilities of Children with Williams Syndrome

Science.gov (United States)

Rossi, Natalia F.; Heinze, Elena Garayzabal; Giacheti, Celia M.; Goncalves, Oscar F.; Sampaio, Adriana

2012-01-01

The objective of this study was to investigate the psycholinguistic abilities of children with Williams syndrome (WS) and typically developing children using the Illinois Test of Psycholinguistic Abilities (ITPA). Performance on the ITPA was analysed in a group with WS (N=20, mean age=8.5 years, SD=1.62) and two typically developing groups,…

Neurocranial morphology and growth in Williams syndrome.

Science.gov (United States)

Axelsson, Stefan; Kjaer, Inger; Heiberg, Arvid; Bjørnland, Tore; Storhaug, Kari

2005-02-01

Williams syndrome (WS) is a rare congenital neurodevelopmental disorder with distinctive facial features, cardiovascular abnormalities, short stature, mental retardation, and behaviour and cognitive characteristics. The aim of this study was to describe the neurocranial morphology and growth in a group of 62 individuals with WS. The neurocranium was analysed on lateral cephalograms and comparisons were made with neurocranial standards from longitudinal data derived from the Oslo University Craniofacial Growth Archive. The size and morphology of the neurocranium in WS subjects differed from controls. Females as a group showed greater differences than males. The posterior cranial base length was shorter in both WS males and females, and the anterior cranial base length was shorter in WS females whereas it was close to normal in the WS male group. The cranial base angle was, however, not different from the control groups. A flattening was seen in the superior aspect of the parietal bone in both WS males and females. In the posterior part of the neurocranium, the prominence of the occipital bone was larger than in the control groups, which was also reflected in a larger total length of the neurocranium. The thickness of the frontal and occipital bones was considerably greater than in the control group. The deviant size and morphology of the neurocranium in WS subjects was already established in the youngest age group and maintained throughout the observation period. The growth pattern of the neurocranium in WS subjects seemed to be similar to that of the control groups, except in a few individuals.

What motion is: William Neile and the laws of motion.

Science.gov (United States)

Kemeny, Max

2017-07-01

In 1668-1669 William Neile and John Wallis engaged in a protracted correspondence regarding the nature of motion. Neile was unhappy with the laws of motion that had been established by the Royal Society in three papers published in 1668, deeming them not explanations of motion at all, but mere descriptions. Neile insisted that science could not be informative without a discussion of causes, meaning that Wallis's purely kinematic account of collision could not be complete. Wallis, however, did not consider Neile's objections to his work to be serious. Rather than engage in a discussion of the proper place of natural philosophy in science, Wallis decided to show how Neile's preferred treatment of motion lead to absurd conclusions. This dispute is offered as a case study of dispute resolution within the early Royal Society.

78 FR 52169 - Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan...

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2013-08-22

... DEPARTMENT OF EDUCATION [Docket No. ED-2013-ICCD-0109] Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan (Direct Loan) Program Federal Direct PLUS Loan Master... to this notice will be considered public records. Title of Collection: William D. Ford Federal Direct...

Performance on verbal and low-verbal false belief tasks: evidence from children with Williams syndrome.

Science.gov (United States)

Van Herwegen, Jo; Dimitriou, Dagmara; Rundblad, Gabriella

2013-01-01

Previous studies that have investigated the relationship between performance on theory of mind (ToM) tasks and verbal abilities in individuals with Williams syndrome (WS) have reported contradictory findings with some showing that language abilities aid performance on ToM tasks while others have found that participants with WS fail these tasks because of their verbal demands. The current study investigated this relationship again comparing performance on a classical change-location task to two newly developed low-verbal tasks, one change-location task and one unexpected content task. Thirty children with WS (aged 5-17;01 years) and 30 typically developing (TD) children (aged between 2;10 years and 9;09 years), who were matched for vocabulary comprehension scores were included in the study. Although performance in the WS group was significantly poorer compared to the TD group on all three tasks, performance was not predicted by their receptive vocabulary or grammatical ability scores. In addition, ToM abilities in both groups depended on the cognitive demands of the task at hand. This finding shows that performance on ToM tasks in WS is not necessarily hindered by their delayed language abilities but rather by the task administered. This could potentially affect the diagnosis of developmental disorders, such as Autism Spectrum Disorders, and comparison of ToM abilities across developmental disorders. Readers of this article should be able to (1) describe the current state of theory of mind research in Williams syndrome, (2) identify which cognitive abilities might explain performance on theory of mind tasks in both typically developing children and in children with Williams syndrome, and (3) interpret the importance of task demands when assessing children's theory of mind abilities. Copyright © 2013 Elsevier Inc. All rights reserved.

Williams syndrome and mature B-Leukemia: A random association?

Science.gov (United States)

Decimi, Valentina; Fazio, Grazia; Dell'Acqua, Fabiola; Maitz, Silvia; Galbiati, Marta; Rizzari, Carmelo; Biondi, Andrea; Cazzaniga, Giovanni; Selicorni, Angelo

2016-12-01

Williams syndrome (WBS) is a rare neurodevelopmental disorder with specific phenotypic characteristics and cardiac abnormalities, but is not considered as a cancer predisposing condition. However, in rare cases, malignancies have been described in patients with WBS, with hematologic cancer (mainly Burkitt Lymphoma and Acute Lymphoblastic Leukemia) as the most represented. We report here the case of a boy with WS and B-NHL. This is the unique case within the large cohort of patients (n = 117) followed in our institution for long time (mean clinical follow-up, 13 years). We herewith propose that the BCL7B gene, located in the chromosomal region commonly deleted in Williams syndrome, could potentially have a role in this particular association. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Ischemic Stroke in Williams-Beuren Syndrome: A Case Report

Directory of Open Access Journals (Sweden)

Wei-Der Lee

2009-04-01

Full Text Available A 15-year-old girl was admitted because of an acute onset of facial palsy and right hemiparesis. The patient had a history of moderate mental retardation and developmental delay. On admission, her vital signs were stable, except for high blood pressure. Magnetic resonance imaging demonstrated an infarct involving the left internal capsule and putamen. Because of the patient's young age, an extensive stroke survey was performed. Williams-Beuren syndrome was finally confirmed by fluorescent in situ hybridization. Compared with the previously reported cases, no evidence of cerebral arterial stenosis or cardiac abnormalities was found by noninvasive imaging techniques. Because Williams-Beuren syndrome is a complex, multiple congenital anomaly syndrome with prominent cardiovascular features, regular assessment and antihypertensive treatment are necessary to minimize the lifelong cardiovascular risk in patients with this syndrome.

Vernacular Authorship in Late Medieval Religious Discourse. The Case of William Flete’s Remedies against Temptations

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Gabriella Del Lungo Camiciotti

2012-03-01

Full Text Available Despite the fact that the main topic of William Flete’s Remedies against Temptations was a pivotal concern of late medieval spiritual literature and the treatise in letter form was widely circulated in both Latin and English, it has remained rather marginal to critical discourse. Neither epistolary space as the site of interaction author/audience nor the role of spiritual authorities in establishing themselves as real authors of religious texts as distinguished from compilers and scribes have been specifically investigated. The paper focuses on the dialogic construction of the authorial voice in William Flete’s Remedies against Temptations through the analysis of the linguistic and discursive strategies used in the vernacular version of this work of spiritual advice. The most relevant strategy is the choice of the letter format to address a female audience as it allows to transfer authoritative religious discourse into English and to assert the writer’s status of author of a text addressed to both religious women and the lay public. In addition, the paper aims at highlighting the relevance of stylistic analysis to delineate the construction of the textual vernacular author in the context of audience recognition. In addressing a non-academic public, the author of one of the English versions of Remedies against Temptations engages with Latin learning and asserts himself as the author of a vernacular theology text.

False belief attribution in children with Williams syndrome: the answer is in the emotion.

Science.gov (United States)

Campos, R; Martínez-Castilla, P; Sotillo, M

2017-11-01

Individuals with Williams syndrome (WS) show difficulties in attributing false beliefs, whereas they are better at attributing emotions. This study examines whether being asked about the emotion linked to a false belief, instead of explicitly about the belief, facilitates performance on theory of mind (ToM) tasks. Thirty children with WS and 90 typically developing children, who were individually matched on mental age (50-112 months), were administered six explicit (i.e. questions on belief) and six implicit (i.e. questions on emotion) trials of false belief tasks. Theory of mind competences were related to cognitive development. Children with WS performed comparably to typically developing children on the emotion questions. Correct answers to questions on emotion reveal an implicit understanding of false belief. The Representational redescription process could be impaired in the domain of ToM in this population. This finding has relevant implications for the design of supports aiming to optimise the development of ToM competences in individuals with WS. © 2017 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

Conceptualizing neurodevelopmental disorders through a mechanistic understanding of fragile X syndrome and Williams syndrome.

Science.gov (United States)

Fung, Lawrence K; Quintin, Eve-Marie; Haas, Brian W; Reiss, Allan L

2012-04-01

The overarching goal of this review is to compare and contrast the cognitive-behavioral features of fragile X syndrome (FraX) and Williams syndrome and to review the putative neural and molecular underpinnings of these features. Information is presented in a framework that provides guiding principles for conceptualizing gene-brain-behavior associations in neurodevelopmental disorders. Abnormalities, in particular cognitive-behavioral domains with similarities in underlying neurodevelopmental correlates, occur in both FraX and Williams syndrome including aberrant frontostriatal pathways leading to executive function deficits, and magnocellular/dorsal visual stream, superior parietal lobe, inferior parietal lobe, and postcentral gyrus abnormalities contributing to deficits in visuospatial function. Compelling cognitive-behavioral and neurodevelopmental contrasts also exist in these two disorders, for example, aberrant amygdala and fusiform cortex structure and function occurring in the context of contrasting social behavioral phenotypes, and temporal cortical and cerebellar abnormalities potentially underlying differences in language function. Abnormal dendritic development is a shared neurodevelopmental morphologic feature between FraX and Williams syndrome. Commonalities in molecular machinery and processes across FraX and Williams syndrome occur as well - microRNAs involved in translational regulation of major synaptic proteins; scaffolding proteins in excitatory synapses; and proteins involved in axonal development. Although the genetic variations leading to FraX and Williams syndrome are different, important similarities and contrasts in the phenotype, neurocircuitry, molecular machinery, and cellular processes in these two disorders allow for a unique approach to conceptualizing gene-brain-behavior links occurring in neurodevelopmental disorders.

What does Williams Syndrome Reveal about the Determinants of Social Behavior?

Directory of Open Access Journals (Sweden)

Anna Maaria Järvinen

2013-06-01

Full Text Available Growing evidence on autonomic nervous system (ANS function in individuals with Williams syndrome (WS has begun to highlight aberrancies that may have important implications for the social profile characterized by enhanced social motivation and approach. In parallel, neurobiological investigations have identified alterations in the structure, function, and connectivity of the amygdala, as well as prosocial neuropeptide dysregulation, as some of the key neurogenetic features of WS. A recent social approach/withdrawal hypothesis (Kemp and Guastella, 2011 suggests that autonomic cardiac control may play a key role in regulating the relationship between oxytocin and social behavior. This article discusses evidence from these critical, new strands of research into social behavior in WS, to consider the extent to which data on WS may provide novel insight into the determinants of social behavior. Future research directions are suggested.

Anxiety and Repetitive Behaviours in Autism Spectrum Disorders and Williams Syndrome: A Cross-Syndrome Comparison

Science.gov (United States)

Rodgers, Jacqui; Riby, Deborah M.; Janes, Emily; Connolly, Brenda; McConachie, Helen

2012-01-01

Children with Autism Spectrum Disorder or Williams syndrome are vulnerable to anxiety. The factors that contribute to this risk remain unclear. This study compared anxiety in autism spectrum disorder and Williams Syndrome and examined the relationship between repetitive behaviours and anxiety. Thirty-four children with autism and twenty children…

Electrophysiological Correlates of Semantic Processing in Williams Syndrome

Science.gov (United States)

Pinheiro, Ana P.; Galdo-Alvarez, Santaigo; Sampaio, Adriana; Niznikiewicz, Margaret; Goncalves, Oscar F.

2010-01-01

Williams syndrome (WS), a genetic neurodevelopmental disorder due to microdeletion in chromosome 7, has been described as a syndrome with an intriguing socio-cognitive phenotype. Cognitively, the relative preservation of language and face processing abilities coexists with severe deficits in visual-spatial tasks, as well as in tasks involving…

Non-Traditional Authorship Attribution Studies of William Shakespeare’s Canon: Some Caveats

Directory of Open Access Journals (Sweden)

Joseph Rudman

2016-03-01

Full Text Available The paper looks at the problems in conducting non-traditional authorship attribution studies on the canon of William Shakespeare. After a short introduction, the case is put forth that these studies are ‘scientific’ and must adhere to the tenets of the scientific method. By showing that a complete and valid experimental plan is necessary and pointing out the many and varied pitfalls (e.g., the text, the control groups, the treatment of errors, it becomes clear what a valid study of Shakespearean non-traditional authorship attribution demands. I then come to the conclusion that such a valid study is not attainable with the limits of present-day knowledge.

Repetition Priming in Adults with Williams Syndrome: Age-Related Dissociation between Implicit and Explicit Memory

Science.gov (United States)

Krinsky-McHale, Sharon J.; Kittler, Phyllis; Brown, W. Ted; Jenkins, Edmund C.; Devenny, Darlynne A.

2005-01-01

We examined implicit and explicit memory in adults with Williams syndrome. An age-related dissociation was found; repetition priming (reflecting implicit memory) did not show change with age, but free recall (reflecting explicit memory) was markedly reduced. We also compared the performance of adults with Williams syndrome to adults with Down…

Addressing social skills deficits in adults with Williams syndrome.

Science.gov (United States)

Fisher, Marisa H; Morin, Lindsay

2017-12-01

Individuals with Williams syndrome (WS) are hypersocial; yet, they experience social difficulties and trouble with relationships. This report summarizes findings from three studies examining the social functioning of adults with WS and the feasibility of a social skills training program for adults with WS (SSTP-WS) through the examination of performance on initial lesson plans. Study 1: 114 parents of adults with WS completed the Social Responsiveness Scale-2. Study 2: 10 adults with WS and 12 of their parents participated in focus groups to further describe the deficits identified in Study 1 and to discuss a SSTP-WS. Study 3: 30 adults with WS were randomly assigned to 2 lessons on either conversations or relationships and pre-post change in social skills knowledge was assessed. Study 1 indicates adults with WS experience severe social impairments in social cognition, and mild-moderate impairments in social awareness and social communication. Qualitative results in Study 2 indicate a SSTP-WS should address conversation skills and relationships. In Study 3, participants showed gains in social skills knowledge following completion of lessons. A SSTP-WS may be beneficial for adults with WS. Future research should describe the social needs of individuals with WS at different ages and should further develop a SSTP-WS. Copyright © 2017 Elsevier Ltd. All rights reserved.

Neuron density is decreased in the prefrontal cortex in Williams syndrome.

Science.gov (United States)

Lew, Caroline Horton; Brown, Chelsea; Bellugi, Ursula; Semendeferi, Katerina

2017-01-01

Williams Syndrome (WS) is a rare neurodevelopmental disorder associated with a hemideletion in chromosome 7, which manifests a distinct behavioral phenotype characterized by a hyperaffiliative social drive, in striking contrast to the social avoidance behaviors that are common in Autism Spectrum Disorder (ASD). MRI studies have observed structural and functional abnormalities in WS cortex, including the prefrontal cortex (PFC), a region implicated in social cognition. This study utilizes the Bellugi Williams Syndrome Brain Collection, a unique resource that comprises the largest WS postmortem brain collection in existence, and is the first to quantitatively examine WS PFC cytoarchitecture. We measured neuron density in layers II/III and V/VI of five cortical areas: PFC areas BA 10 and BA 11, primary motor BA 4, primary somatosensory BA 3, and visual area BA 18 in six matched pairs of WS and typically developing (TD) controls. Neuron density in PFC was lower in WS relative to TD, with layers V/VI demonstrating the largest decrease in density, reaching statistical significance in BA 10. In contrast, BA 3 and BA 18 demonstrated a higher density in WS compared to TD, although this difference was not statistically significant. Neuron density in BA 4 was similar in WS and TD. While other cortical areas were altered in WS, prefrontal areas appeared to be most affected. Neuron density is also altered in the PFC of individuals with ASD. Together these findings suggest that the PFC is targeted in neurodevelopmental disorders associated with sociobehavioral alterations. Autism Res 2017, 10: 99-112. © 2016 International Society for Autism Research, Wiley Periodicals, Inc. © 2016 International Society for Autism Research, Wiley Periodicals, Inc.

Williams syndrome predisposes to vascular stiffness modified by antihypertensive use and copy number changes in NCF1.

Science.gov (United States)

Kozel, Beth A; Danback, Joshua R; Waxler, Jessica L; Knutsen, Russell H; de Las Fuentes, Lisa; Reusz, Gyorgy S; Kis, Eva; Bhatt, Ami B; Pober, Barbara R

2014-01-01

Williams syndrome is caused by the deletion of 26 to 28 genes, including elastin, on human chromosome 7. Elastin insufficiency leads to the cardiovascular hallmarks of this condition, namely focal stenosis and hypertension. Extrapolation from the Eln(+/-) mouse suggests that affected people may also have stiff vasculature, a risk factor for stroke, myocardial infarction, and cardiac death. NCF1, one of the variably deleted Williams genes, is a component of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex and is involved in the generation of oxidative stress, making it an interesting candidate modifier for vascular stiffness. Using a case-control design, vascular stiffness was evaluated by pulse wave velocity in 77 Williams cases and matched controls. Cases had stiffer conducting vessels than controls (PWilliams syndrome. Pulse wave velocity increased with age at comparable rates in cases and controls, and although the degree of vascular stiffness varied, it was seen in both hypertensive and normotensive Williams participants. Use of antihypertensive medication and extension of the Williams deletion to include NCF1 were associated with protection from vascular stiffness. These findings demonstrate that vascular stiffness is a primary vascular phenotype in Williams syndrome and that treatment with antihypertensives or agents inhibiting oxidative stress may be important in managing patients with this condition, potentially even those who are not overtly hypertensive.

Potential Beneficial Effects of Tulbaghia violacea William Henry ...

African Journals Online (AJOL)

Tulbaghia violacea William Henry Harvey (Harv. Alliaceae) is a small bulbous herb belonging to the family Alliaceae. It is used in South Africa to treat fever, colds, asthma, paralysis, and hypertension. Meanwhile, cardiovascular disease accounts for about 30 % of total global death, with most of these deaths occurring in low ...

Alternative treatment for open bite Class III malocclusion in a child with Williams-Beuren syndrome

Directory of Open Access Journals (Sweden)

Giovanni Modesto Vieira

2015-02-01

Full Text Available Williams-Beuren syndrome (WBS is a rare genetic condition that affects approximately 1 in every 20,000 - 50,000 live births. WBS children have specific skeletal deformities, dental malformations and rare lingual muscle dysfunction. The need for orthodontic and orthognathic therapy has arisen and has been considered a real clinical challenge even for experienced professionals, once it requires a complex and individualized treatment plan. This study reports a case of orthopedic expansion of the maxilla, in which a modified facial mask was used for protraction of the maxillary complex associated with clockwise rotation of the maxilla. In addition, special considerations about treatment time and orthopedic outcomes are discussed.

77 FR 58818 - Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal...

Science.gov (United States)

2012-09-24

... DEPARTMENT OF EDUCATION Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal Direct Loan Program Regulations-- Servicemembers Civil Relief Act SUMMARY: Upon a... in response to this notice will be considered public records. Title of Collection: William D. Ford...

Estrutura e significado em "Uma rosa para Emily", de William Faulkner

Directory of Open Access Journals (Sweden)

Carlos Daghlian

2004-01-01

Full Text Available Trata-se de uma análise do consagrado conto "Uma Rosa para Emily", de William Faulkner, voltada para alguns dos principais aspectos de sua estrutura. Após considerarmos o enredo, discutimos a construção das personagens, com destaque para a protagonista, fazendo um levantamento e comentários sobre possíveis fontes de inspiração, destacando, entre outras, aspectos da biografia da poeta Emily Dickinson, a ficção e a poesia de E. A. Poe, romances de Charles Dickens e Henry James, o conto de Sherwood Anderson e a poesia de William Blake, Emily Dickinson, Robert Browning e John Crowe Ransom, acrescentando paralelos com o conto "Bartleby, o escrivão", de Herman Melville. Analisamos, então, o foco narrativo, os símbolos e o significado, ressaltando aqui o desenvolvimento temático da narrativa.This is an analysis of the well-known short story "A Rose for Emily," by William Faulkner, concentrating on some of the main aspects of its structure. A consideration of the plot is followed by a discussion of characterization, with emphasis on the protagonist, by means of a survey and comments on possible sources of inspiration including, among others, aspects of Emily Dickinson's biography, E. A. Poe's fiction and poetry, novels by Charles Dickens and Henry James, Sherwood Anderson's short stories, and the poetry of William Blake, Emily Dickinson, Robert Browning, and John Crowe Ransom, in addition to Herman Melville's short story "Bartleby, the Scrivener." The narrative focus, symbolism and meaning, stressing the thematic development of the narrative, are then analyzed.

Utility of the Hebb-Williams Maze Paradigm for Translational Research in Fragile X Syndrome: A Direct Comparison of Mice and Humans.

Science.gov (United States)

Boutet, Isabelle; Collin, Charles A; MacLeod, Lindsey S; Messier, Claude; Holahan, Matthew R; Berry-Kravis, Elizabeth; Gandhi, Reno M; Kogan, Cary S

2018-01-01

To generate meaningful information, translational research must employ paradigms that allow extrapolation from animal models to humans. However, few studies have evaluated translational paradigms on the basis of defined validation criteria. We outline three criteria for validating translational paradigms. We then evaluate the Hebb-Williams maze paradigm (Hebb and Williams, 1946; Rabinovitch and Rosvold, 1951) on the basis of these criteria using Fragile X syndrome (FXS) as model disease. We focused on this paradigm because it allows direct comparison of humans and animals on tasks that are behaviorally equivalent (criterion #1) and because it measures spatial information processing, a cognitive domain for which FXS individuals and mice show impairments as compared to controls (criterion #2). We directly compared the performance of affected humans and mice across different experimental conditions and measures of behavior to identify which conditions produce comparable patterns of results in both species. Species differences were negligible for Mazes 2, 4, and 5 irrespective of the presence of visual cues, suggesting that these mazes could be used to measure spatial learning in both species. With regards to performance on the first trial, which reflects visuo-spatial problem solving, Mazes 5 and 9 without visual cues produced the most consistent results. We conclude that the Hebb-Williams mazes paradigm has the potential to be utilized in translational research to measure comparable cognitive functions in FXS humans and animals (criterion #3).

Bayesian stock assessment of Pacific herring in Prince William Sound, Alaska.

Science.gov (United States)

Muradian, Melissa L; Branch, Trevor A; Moffitt, Steven D; Hulson, Peter-John F

2017-01-01

The Pacific herring (Clupea pallasii) population in Prince William Sound, Alaska crashed in 1993 and has yet to recover, affecting food web dynamics in the Sound and impacting Alaskan communities. To help researchers design and implement the most effective monitoring, management, and recovery programs, a Bayesian assessment of Prince William Sound herring was developed by reformulating the current model used by the Alaska Department of Fish and Game. The Bayesian model estimated pre-fishery spawning biomass of herring age-3 and older in 2013 to be a median of 19,410 mt (95% credibility interval 12,150-31,740 mt), with a 54% probability that biomass in 2013 was below the management limit used to regulate fisheries in Prince William Sound. The main advantages of the Bayesian model are that it can more objectively weight different datasets and provide estimates of uncertainty for model parameters and outputs, unlike the weighted sum-of-squares used in the original model. In addition, the revised model could be used to manage herring stocks with a decision rule that considers both stock status and the uncertainty in stock status.

Bayesian stock assessment of Pacific herring in Prince William Sound, Alaska.

Directory of Open Access Journals (Sweden)

Melissa L Muradian

Full Text Available The Pacific herring (Clupea pallasii population in Prince William Sound, Alaska crashed in 1993 and has yet to recover, affecting food web dynamics in the Sound and impacting Alaskan communities. To help researchers design and implement the most effective monitoring, management, and recovery programs, a Bayesian assessment of Prince William Sound herring was developed by reformulating the current model used by the Alaska Department of Fish and Game. The Bayesian model estimated pre-fishery spawning biomass of herring age-3 and older in 2013 to be a median of 19,410 mt (95% credibility interval 12,150-31,740 mt, with a 54% probability that biomass in 2013 was below the management limit used to regulate fisheries in Prince William Sound. The main advantages of the Bayesian model are that it can more objectively weight different datasets and provide estimates of uncertainty for model parameters and outputs, unlike the weighted sum-of-squares used in the original model. In addition, the revised model could be used to manage herring stocks with a decision rule that considers both stock status and the uncertainty in stock status.

Neural Correlates of Amusia in Williams Syndrome

OpenAIRE

Lense, Miriam D.; Dankner, Nathan; Pryweller, Jennifer R.; Thornton-Wells, Tricia A.; Dykens, Elisabeth M.

2014-01-01

Congenital amusia is defined by marked deficits in pitch perception and production. Though historically examined only in otherwise typically developing (TD) populations, amusia has recently been documented in Williams syndrome (WS), a genetic, neurodevelopmental disorder with a unique auditory phenotype including auditory sensitivities and increased emotional responsiveness to music but variable musical skill. The current study used structural T1-weighted magnetic resonance imaging and diffus...

76 FR 13667 - Commercial Furniture Group, Inc., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe...

Science.gov (United States)

2011-03-14

... Furniture Group, Inc., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe and Thonet, Including...., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe and Thonet, Chicago, IL; Amended Certification Regarding Eligibility To Apply for Worker Adjustment Assistance In accordance with Section 223 of...

Report of Some Comets: The Discovery of Uranus and Comets by William, Caroline, and John Herschel

Science.gov (United States)

Pasachoff, Jay M.; Olson, R. J. M.

2011-01-01

We report on the discovery and drawings of comets by William, Caroline, and John Herschel. The first discovery, by William Herschel, in 1781 from Bath, published in the Philosophical Transactions of the Royal Society with the title "Report of a Comet," turned out to be Uranus, the first planet ever discovered, Mercury through Saturn having been known since antiquity. William's sister Caroline was given duties of sweeping the skies and turned out to be a discoverer of 8 comets in her own right, in addition to keeping William's notes. Caroline's comets were discovered from Slough between 1786 and 1797. In the process, we also discuss original documents from the archives of the Royal Society and of the Royal Astronomical Society. We conclude by showing comet drawings that we have recently attributed to John Herschel, including Halley's Comet from 1836, recently located in the Ransom Center of the University of Texas at Austin. Acknowledgments: Planetary astronomy at Williams College is supported in part by grant NNX08AO50G from NASA Planetary Astronomy. We thank Peter Hingley of the Royal Astronomical Society and Richard Oram of the Harry Ransom Center of The University of Texas at Austin for their assistance.

Are the deficits in navigational abilities present in the Williams syndrome related to deficits in the backward inhibition?

Directory of Open Access Journals (Sweden)

Francesca eFoti

2015-03-01

Full Text Available Williams syndrome (WS is associated with a distinct profile of relatively proficient skills within the verbal domain compared to the severe impairment of visuo-spatial processing. Abnormalities in executive functions and deficits in planning ability and spatial working memory have been described. However, to date little is known about the influence of executive function deficits on navigational abilities in WS. This study aimed at analyzing in WS individuals a specific executive function, the backward inhibition (BI that allows individuals to flexibly adapt to continuously changing environments. A group of WS individuals and a mental age- and gender-matched group of typically developing (TD children were subjected to three task-switching experiments requiring visuospatial or verbal material to be processed. Results showed that WS individuals exhibited clear BI deficits during visuospatial task-switching paradigms and normal BI effect during verbal task-switching paradigm. Overall, the present results suggest that the BI involvement in updating environment representations during navigation may influence WS navigational abilities.

Prince William Sound, Alaska ESI: M_MAMMAL (Marine Mammal Polygons)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

WE-G-213-02: The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who Were They and What Did They Do?

Energy Technology Data Exchange (ETDEWEB)

Rothenberg, L. [Memorial Sloan-Kettering Cancer Center (United States)

2015-06-15

Roentgen and the Birth of Modern Medical Physics – Perry Sprawls Wilhelm Roentgen is well known for his discovery of x-radiation. What is less known and appreciated is his intensive research following the discovery to determine the characteristics of the “new kind of radiation” and demonstrate its great value for medical purposes. In this presentation we will imagine ourselves in Roentgen’s mind and follow his thinking, including questions and doubts, as he designs and conducts a series of innovative experiments that provided the foundation for the rapid growth of medical physics. Learning Objectives: Become familiar with the personal characteristics and work of Prof. Roentgen that establishes him as an inspiring model for the medical physics profession. Observe the thought process and experiments that determined and demonstrated the comprehensive characteristics of x-radiation. The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who were they and what did they do? – Lawrence N. Rothenberg William David Coolidge (1873–1975) William Coolidge was born in Hudson, NY in 1873. He obtained his BS at the Massacusetts Institute of Technology in 1896. Coolidge then went to the University of Leipzig, Germany for graduate study with physicists Paul Drude and Gustave Wiedemann and received a Ph.D. in 1899. While in Germany he met Wilhelm Roentgen. Coolidge returned to the US to teach at MIT where he was associated with Arthur A. Noyes of the Chemistry Department, working on the electrical conductivity of aqueous solutions. Willis R. Whitney, under whom Coolidge had worked before going to Germany, became head of the newly formed General Electric Research Laboratory and he invited Coolidge to work with him. In 1905, Coolidge joined the staff of the GE laboratory and was associated with it for the remainder of his life. He developed ductile tungsten filaments to replace fragile carbon filaments as the material for electric light

WE-G-213-02: The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who Were They and What Did They Do?

International Nuclear Information System (INIS)

Rothenberg, L.

2015-01-01

Roentgen and the Birth of Modern Medical Physics – Perry Sprawls Wilhelm Roentgen is well known for his discovery of x-radiation. What is less known and appreciated is his intensive research following the discovery to determine the characteristics of the “new kind of radiation” and demonstrate its great value for medical purposes. In this presentation we will imagine ourselves in Roentgen’s mind and follow his thinking, including questions and doubts, as he designs and conducts a series of innovative experiments that provided the foundation for the rapid growth of medical physics. Learning Objectives: Become familiar with the personal characteristics and work of Prof. Roentgen that establishes him as an inspiring model for the medical physics profession. Observe the thought process and experiments that determined and demonstrated the comprehensive characteristics of x-radiation. The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who were they and what did they do? – Lawrence N. Rothenberg William David Coolidge (1873–1975) William Coolidge was born in Hudson, NY in 1873. He obtained his BS at the Massacusetts Institute of Technology in 1896. Coolidge then went to the University of Leipzig, Germany for graduate study with physicists Paul Drude and Gustave Wiedemann and received a Ph.D. in 1899. While in Germany he met Wilhelm Roentgen. Coolidge returned to the US to teach at MIT where he was associated with Arthur A. Noyes of the Chemistry Department, working on the electrical conductivity of aqueous solutions. Willis R. Whitney, under whom Coolidge had worked before going to Germany, became head of the newly formed General Electric Research Laboratory and he invited Coolidge to work with him. In 1905, Coolidge joined the staff of the GE laboratory and was associated with it for the remainder of his life. He developed ductile tungsten filaments to replace fragile carbon filaments as the material for electric light

The world oil market is not one great pool: A reply to Rodriguez and Williams

International Nuclear Information System (INIS)

Weiner, R.J.

1993-01-01

In a paper by Rodriguez and Williams (Energy Studies Review, 1993), the case for a single-pool world oil market is put forward. Criticisms of the assumptions and conclusions are offered. To counter the paper's assumption that antitrust is the relevant metric for measuring the extent of geographic markets, it is argued that for energy security policy decisions, it is the economic market which matters. The paper does show that the world oil market is indeed unified in the long run, but this is not relevant to the short term, the relevant time-frame for energy security decisions. The paper's choice of spot market data for its cointegration analysis is also criticized by using several examples of pricing decisions that would not make sense in a unified world market but which do make sense in a regionalized market. It is also unclear how representative spot prices are of the oil market. Finally, the conclusions of the paper that favor policies focusing on secure vs insecure import sources and supplier diversification are seen as unsupported by the paper's statistical findings. 15 refs

Edward Christopher Williams and His Impact on Librarianship.

Science.gov (United States)

Latimer, Carlos

Edward Christopher Williams had a major impact on librarianship, not only as the first documented African American to graduate from a library school, but also as a developer of education for librarians and as an active member of the American Library Association (ALA) and the Ohio Library Association. This study used the historical methodology…

Histopathology of adult Pacific herring in Prince William Sound, Alaska, after the Exxon Valdez oil spill

International Nuclear Information System (INIS)

Marty, G. S.; Okihiro, M. S.; Hinton, D. E.; Brown, E. D.; Hanes, D.

1999-01-01

The histopathology of Pacific herring sampled from oiled sites in Prince William Sound, Alaska, three weeks after the 1989 Exxon Valdez oil spill, is discussed. All samples showed multifocal hepatic necrosis and significantly increased tissue concentrations of polynuclear aromatic hydrocarbons (PAH). In contrast, Pacific herring from reference sites in 1989 and from all sites in 1990 and 1991 did not have hepatic necrosis or increase PAH concentrations. Naphthalenes were the predominant PAH in all tissue samples. The development of hepatic necrosis and the predominance of naphthalenes in samples from 1989 is considered consistent with results obtained from recent laboratory studies indicating that crude oil exposure resulted in dose-dependent expression of viral hemorrhagic septicemia virus (VHSV). It was concluded that adult Pacific herring in Prince William Sound were likely exposed to Exxon Valdez oil in April 1989. Because Pacific herring near spawning condition in early spring are in a state of physiological stress, the added stress of oil exposure in 1989 could reasonably have led to expression of VHSV in these fish. The findings suggest that the immediate response to future large toxic spills should include consideration of the potential interaction of multiple stressors in exposed individuals. 29 refs., 7 tabs., 1 fig

Two Thinking Skills Assessment Approaches: "Assessment of Pupils' Thinking Skills" and "Individual Thinking Skills Assessments"

Science.gov (United States)

Burke, Lynsey A.; Williams, Joanne M.

2012-01-01

This paper is linked to a previous paper outlining an evaluation of a thinking skills intervention (Burke & Williams, 2008). Following extensive requests for the assessment tools used in the intervention, this short paper presents the development and potential uses of two thinking skills assessment tools. The aim of the paper is simply to make…

Health and Social Outcomes in Adults with Williams Syndrome: Findings from Cross-Sectional and Longitudinal Cohorts

Science.gov (United States)

Elison, Sarah; Stinton, Chris; Howlin, Patricia

2010-01-01

Previous studies have investigated trajectories of cognitive, language and adaptive functioning in Williams syndrome (WS) but little is known about how other aspects of the Williams syndrome behavioural phenotype change across the life-span. Therefore, the present study examined age associated changes in a number of different domains of…

Comparison of the acute effects of Tulbaghia violacea William Henry ...

African Journals Online (AJOL)

William Henry Harvey (Alliaceae) on blood pressure and heart rate of ... (WKY), and compare the results obtained with those for adult spontaneously hypertensive rats (SHR). Methods: T. ... have similar secondary metabolites and biological ...

Continuous cognitive dynamics of the evaluation of trustworthiness in Williams syndrome

Directory of Open Access Journals (Sweden)

Marilee A Martens

2012-06-01

Full Text Available The decision to approach or avoid an unfamiliar person is based in part on one’s evaluation of facial expressions. Individuals with Williams syndrome (WS are characterized in part by an excessive desire to approach people, but they display deficits in identifying facial emotional expressions. Likert-scale ratings are generally used to examine approachability ratings in WS, but these measures only capture an individual’s final approach/avoid decision. The present study expands on previous research by utilizing mouse-tracking methodology to visually display the nature of approachability decisions via the motor movement of a computer mouse. We recorded mouse movement trajectories while participants chose to approach or avoid computer-generated faces that varied in terms of trustworthiness. We recruited 30 individuals with WS and 30 chronological age-matched controls (mean age = 20 years. Each participant performed 80 trials (20 trials each of four face types: mildly and extremely trustworthy; mildly and extremely untrustworthy. We found that individuals with WS were significantly more likely than controls to choose to approach untrustworthy faces. In addition, the maximum deviation analysis demonstrated that even when ultimately choosing to avoid an untrustworthy face, the individuals with WS were more tempted than controls to deviate toward ‘Approach’ before deciding to ‘Avoid’ the face. We also found that the individuals with WS were more likely to choose to avoid trustworthy faces than controls, which might be associated with the neutral facial expressions displayed by the trustworthy faces. Both the WS and control participants were able to discriminate between mild and extreme degrees of trustworthiness and were more likely to make correct approachability decisions as they grew older. These findings increase our understanding of the cognitive processing that underlies approachability decisions in individuals with WS.

ProRisk : risk analysis instrument : developed for William properties

NARCIS (Netherlands)

van Doorn, W.H.W.; Egeberg, Ingrid; Hendrickx, Kristoff; Kahramaner, Y.; Masseur, B.; Waijers, Koen; Weglicka, K.A.

2005-01-01

This report presents a Risk Analysis Instrument developed for William Properties. Based on the analysis, it appears that the practice of Risk Analysis exists within the organization, yet rather implicit. The Risk Analysis Instrument comes with a package of four components: an activity diagram, a

William Ospina y la búsqueda de la franja amarilla

Directory of Open Access Journals (Sweden)

Víctor Valero Bernal

2016-12-01

Full Text Available El objetivo de este artículo es el de analizar el ensayo «Lo que le falta a Colombia» del escritor William Ospina. En este ensayo Ospina profundiza en los principales problemas sociales y políticos que Colombia atravesaba a finales del s. XX así como en las posibles soluciones que podrían adoptarse. Los planteamientos que emplea el escritor colombiano pueden relacionarse con una gran variedad de conceptos propios del ámbito de la política y la sociología. A lo largo de este trabajo mostraremos las analogías existentes entre el análisis de William Ospina y las ideas de pensadores tan alejados en el tiempo como Thomas Hobbes, Rousseau, John Rawls o Charles Tilly.

Facial emotion recognition in Williams syndrome and Down syndrome: A matching and developmental study.

Science.gov (United States)

Martínez-Castilla, Pastora; Burt, Michael; Borgatti, Renato; Gagliardi, Chiara

2015-01-01

In this study both the matching and developmental trajectories approaches were used to clarify questions that remain open in the literature on facial emotion recognition in Williams syndrome (WS) and Down syndrome (DS). The matching approach showed that individuals with WS or DS exhibit neither proficiency for the expression of happiness nor specific impairments for negative emotions. Instead, they present the same pattern of emotion recognition as typically developing (TD) individuals. Thus, the better performance on the recognition of positive compared to negative emotions usually reported in WS and DS is not specific of these populations but seems to represent a typical pattern. Prior studies based on the matching approach suggested that the development of facial emotion recognition is delayed in WS and atypical in DS. Nevertheless, and even though performance levels were lower in DS than in WS, the developmental trajectories approach used in this study evidenced that not only individuals with DS but also those with WS present atypical development in facial emotion recognition. Unlike in the TD participants, where developmental changes were observed along with age, in the WS and DS groups, the development of facial emotion recognition was static. Both individuals with WS and those with DS reached an early maximum developmental level due to cognitive constraints.

La Fondation William et Flora Hewlett | IDRC - International ...

International Development Research Centre (IDRC) Digital Library (Canada)

La Fondation William et Flora Hewlett. http://www.hewlett.org/ · What we do · Funding · Resources · About IDRC. Knowledge. Innovation. Solutions. Careers · Contact Us · Site map. Sign up now for IDRC news and views sent directly to your inbox each month. Subscribe · Copyright · Open access policy · Privacy policy ...

Advocating for Inclusion of Children with Williams Syndrome

Science.gov (United States)

Self, Michelle A.

2010-01-01

The purpose of this study was to describe and explore the experience of inclusion of students with Williams syndrome, a rare genetic condition of a microdeletion on chromosome 7 which has medical, behavior, and cognitive issues. The study was conducted by gaining an understanding from the parents' point of view. The study was twofold. First, the…

A Conversation with William A. Fowler Part II

Science.gov (United States)

Greenberg, John

2005-06-01

Physicist William A.Fowler initiated an experimental program in nuclear astrophysics after World War II. He recalls here the Steady State versus Big Bang controversy and his celebrated collaboration with Fred Hoyle and Geoffrey and Margaret Burbidge on nucleosynthesis in stars. He also comments on the shift away from nuclear physics in universities to large accelerators and national laboratories.

[William Harvey revisited ].

Science.gov (United States)

Steinke, Hubert

2015-07-01

William Harvey's discovery of the circulation of the blood is often described as a product of the Scientific Revolution of the Seventeenth Century. Modern research has, however, shown thatHarvey followed the Aristotelian research tradition and thus tried to reveal the purpose of the organs through examination of various animals. His publication of 1628 has to be read as an argument of natural philosophy, or, more precisely, as a series of linked observations, experiments and philosophical reasonings from which the existence of circulation has to be deduced as a logical consequence. Harvey did not consider experiments as superior to philosophical reasoning nor intended he to create a new system of medicine. He believed in the vitality of the heart and the blood and rejected Francis Bacon's empirism and the mechanistic rationalism of Descartes. Harvey's contribution and originality lied less in his single observations and experiments but in the manner how he linked them with critical reasoning and how he accepted, presented and defended the ensuing radical findings.

The role of William Fulbright in the movement against the Vietnam War

Directory of Open Access Journals (Sweden)

Ruth Erken

2009-11-01

Full Text Available El articulo trata de una parte importante de las actividades políticas de Fulbright, es decir su papel en el movimiento en contra de la guerra de Vietnam, que ha recibido relativamente poca atención hasta ahora. El autor de este artículo examina el papel del senador americano William Fulbright en la oposición a la guerra de Vietnam. Para una comprensión correcta de esta actitud es necesario, como se defiende en el texto, conocer la trayectoria, tanto vital como política, de este personaje y considerar el despertar de la conciencia de los americanos con respecto a las políticas nacionales e internacionales. Puesto que es muy difícil establecer claramente una fecha exacta para el brote de la guerra de Vietnam, este papel enfoca en el período entre 1960 y 1970. La política interior americana de los años 60 se debe considerar poco atenta a los considerables desafíos sociales. El movimiento de los derechos civiles, a través del cual se luchó para iguales derechos para las mujeres, los negros, las minorías étnicas y los pobres. Uno de estos movimientos sociales era el movimiento pacifista. Por lo tanto, se estudiarán aquellos movimientos determinantes para la protesta en contra de la guerra en Vietnam, el origen y el desarrollo de estos, las reacciones principales a las misma y el papel que William Fulbright tuvo en la situación política y social tensa que el conflicto proporcionó para los Estados Unidos entre los años 60 y de los años 70._______________ABSTRACT:The present paper, is dedicated to an important part of Fulbright’s political activities, i.e. his role in the movement against the Vietnam war, which has received relatively little attention so far. The author of this article examines the role of the American senator William Fulbright in the opposition to the war of Vietnam. For a correct comprehension of this attitude, it is necessary, as she defends itself in the text, to know the path, so much vitally as politics

Approach to downstream planning for nearshore response and sensitive areas protection outside Prince William Sound, Alaska

International Nuclear Information System (INIS)

DeCola, E.G.; Robertson, T.L.; Robertson, R.; Banta, J.

2004-01-01

This study assessed the need for an oil spill response plan for downstream coastal communities that could be affected by oil spilled from tankers travelling in Prince William Sound, Alaska. For the purpose of oil spill contingency planning, the State of Alaska has been divided into the Kodiak and Cook Inlet sub-areas that are at risk for downstream impacts from a Prince William Sound oil spill. The 1989 Exxon Valdez oil spill provided an example of a worst-case scenario oil spill from a tanker in Prince William Sound, but the oil spill planning system that has evolved in Alaska does not adequately plan for on oil spill that originates in one sub-area of the state, but impacts other sub-areas in the downstream spill path. This study analyzed the gaps that exist in the current response planning system in the Prince William Sound, Cook Inlet and Kodiak sub-areas. A method was proposed to improve the existing response plans so that emergency response teams are better prepared to manage cross-boundary oil spills originating in Prince William Sound. The proposed method focuses on nearshore response and sensitive areas protection for coastlines and communities that are at risk for oil spills from a tanker travelling the Trans-Alaska Pipeline System (TAPS). 11 refs., 3 figs

The developmental trajectory of disruptive behavior in Down syndrome, fragile X syndrome, Prader-Willi syndrome and Williams syndrome.

Science.gov (United States)

Rice, Lauren J; Gray, Kylie M; Howlin, Patricia; Taffe, John; Tonge, Bruce J; Einfeld, Stewart L

2015-06-01

The aim of this study was to investigate the developmental trajectories of verbal aggression, physical aggression, and temper tantrums in four genetic syndrome groups. Participants were part of the Australian Child to Adult Development Study (ACAD), which collected information from a cohort of individuals with an intellectual disability at five time points over 18 years. Data were examined from a total of 248 people with one of the four following syndromes: Down syndrome, Fragile X syndrome, Prader-Willi syndrome, or Williams syndrome. Changes in behaviors were measured using validated items from the Developmental Behavior Checklist (DBC). The results indicate that, while verbal aggression shows no evidence of diminishing with age, physical aggression, and temper tantrums decline with age before 19 years for people with Down syndrome, Fragile X syndrome, and William syndrome; and after 19 years for people with Prader-Willi syndrome. These findings offer a somewhat more optimistic outlook for people with an intellectual disability than has previously been suggested. Research is needed to investigate the mechanisms predisposing people with PWS to persistence of temper tantrums and physical aggression into adulthood. © 2015 Wiley Periodicals, Inc.

Characterisation of Sleep Problems in Children with Williams Syndrome

Science.gov (United States)

Annaz, Dagmara; Hill, Catherine M.; Ashworth, Anna; Holley, Simone; Karmiloff-Smith, Annette

2011-01-01

Sleep is critical to optimal daytime functioning, learning and general health. In children with established developmental disorders sleep difficulties may compound existing learning difficulties. The purpose of the present study was to evaluate the prevalence and syndrome specificity of sleep problems in Williams syndrome (WS), a…

78 FR 63464 - William D. Ford Federal Direct Loan Program Repayment Plan Selection Form; Extension of Public...

Science.gov (United States)

2013-10-24

... DEPARTMENT OF EDUCATION William D. Ford Federal Direct Loan Program Repayment Plan Selection Form; Extension of Public Comment Period; Correction AGENCY: Department of Education. ACTION: Correction notice... entitled, ``William D. Ford Federal Direct Loan Program Repayment Plan Selection Form''. ED is extending...

Pragmatic Abilities of Children with Williams Syndrome: A Longitudinal Examination

Science.gov (United States)

John, Angela E.; Dobson, Lauren A.; Thomas, Lauren E.; Mervis, Carolyn B.

2012-01-01

Prior research has indicated that pragmatics is an area of particular weakness for individuals with Williams syndrome (WS). To further address this aspect of the WS social phenotype, we used an individual differences approach to consider both cross-sectional and longitudinal relations among different pragmatic abilities for 14 children with WS, taking into account individual differences in non-verbal reasoning abilities. We also considered the relations between pragmatic abilities and expressive vocabulary ability. Participants were tested at two time points: as 4-year-olds during a 30-min play session with their mothers (Time 1) and an average of 5.87 years later during a one-on-one conversation with a familiar researcher (Time 2). Children’s intellectual and expressive vocabulary abilities were assessed at both time points. Results indicated that the ability to verbally contribute information beyond what was required in response to a question (ExtendQ) was significantly related to the ability to verbally contribute new information in the absence of a question (ExtendS) both at age 4 years and during primary school. At age 4, both the ability to pair verbalizations with eye contact in triadic interactions (secondary intersubjectivity) and expressive vocabulary ability were related to both ExtendQ and ExtendS. Finally, both ExtendQ and the ability to pair verbalizations with eye contact (intersubjectivity) at age 4 years predicted ExtendQ at age 9–12 years. The theoretical implications of our findings and the importance of early pragmatic language intervention for children who have WS are discussed. PMID:22719734

Utility of the Hebb–Williams Maze Paradigm for Translational Research in Fragile X Syndrome: A Direct Comparison of Mice and Humans

Science.gov (United States)

Boutet, Isabelle; Collin, Charles A.; MacLeod, Lindsey S.; Messier, Claude; Holahan, Matthew R.; Berry-Kravis, Elizabeth; Gandhi, Reno M.; Kogan, Cary S.

2018-01-01

To generate meaningful information, translational research must employ paradigms that allow extrapolation from animal models to humans. However, few studies have evaluated translational paradigms on the basis of defined validation criteria. We outline three criteria for validating translational paradigms. We then evaluate the Hebb–Williams maze paradigm (Hebb and Williams, 1946; Rabinovitch and Rosvold, 1951) on the basis of these criteria using Fragile X syndrome (FXS) as model disease. We focused on this paradigm because it allows direct comparison of humans and animals on tasks that are behaviorally equivalent (criterion #1) and because it measures spatial information processing, a cognitive domain for which FXS individuals and mice show impairments as compared to controls (criterion #2). We directly compared the performance of affected humans and mice across different experimental conditions and measures of behavior to identify which conditions produce comparable patterns of results in both species. Species differences were negligible for Mazes 2, 4, and 5 irrespective of the presence of visual cues, suggesting that these mazes could be used to measure spatial learning in both species. With regards to performance on the first trial, which reflects visuo-spatial problem solving, Mazes 5 and 9 without visual cues produced the most consistent results. We conclude that the Hebb–Williams mazes paradigm has the potential to be utilized in translational research to measure comparable cognitive functions in FXS humans and animals (criterion #3). PMID:29643767

Morphological differences in the mirror neuron system in Williams syndrome.

Science.gov (United States)

Ng, Rowena; Brown, Timothy T; Erhart, Matthew; Järvinen, Anna M; Korenberg, Julie R; Bellugi, Ursula; Halgren, Eric

2016-01-01

Williams syndrome (WS) is a genetic condition characterized by an overly gregarious personality, including high empathetic concern for others. Although seemingly disparate from the profile of autism spectrum disorder (ASD), both are associated with deficits in social communication/cognition. Notably, the mirror neuron system (MNS) has been implicated in social dysfunction for ASD; yet, the integrity of this network and its association with social functioning in WS remains unknown. Magnetic resonance imaging (MRI) methods were used to examine the structural integrity of the MNS of adults with WS versus typically developing (TD) individuals. The Social Responsiveness Scale (SRS), a tool typically used to screen for social features of ASD, was also employed to assess the relationships between social functioning with the MNS morphology in WS participants. WS individuals showed reduced cortical surface area of MNS substrates yet relatively preserved cortical thickness as compared to TD adults. Increased cortical thickness of the inferior parietal lobule (IPL) was associated with increased deficits in social communication, social awareness, social cognition, and autistic mannerisms. However, social motivation was not related to anatomical features of the MNS. Our findings indicate that social deficits typical to both ASD and WS may be attributed to an aberrant MNS, whereas the unusual social drive marked in WS is subserved by substrates distinct from this network.

Anaesthesia-related haemodynamic complications in Williams syndrome patients: a review of one institution's experience.

Science.gov (United States)

Olsen, M; Fahy, C J; Costi, D A; Kelly, A J; Burgoyne, L L

2014-09-01

Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics administered in 29 patients. Twelve of the anaesthetics (11.1%) were associated with cardiac complications including cardiac arrest in two cases (1.85%). Of the two cardiac arrests, one patient died within the first 24 hours postanaesthetic and the other patient survived, giving an overall mortality of 0.9% (3.4%). We conclude that Williams syndrome confers a significant anaesthetic risk, which should be recognised and considered by clinicians planning procedures requiring general anaesthesia.

50 CFR Figure 4 to Subpart E of... - Prince William Sound Rural and Non-Rural Areas

Science.gov (United States)

2010-10-01

... 50 Wildlife and Fisheries 7 2010-10-01 2010-10-01 false Prince William Sound Rural and Non-Rural Areas 4 Figure 4 to Subpart E of Part 300 Wildlife and Fisheries INTERNATIONAL FISHING AND RELATED... to Subpart E of Part 300—Prince William Sound Rural and Non-Rural Areas ER04NO09.010 [74 FR 57110...

The Deconstruction of the Cartesian Dichotomy of Black and Whitein William Blake’s The Little Black Boy

Directory of Open Access Journals (Sweden)

Ali Gunes

2015-12-01

Full Text Available The Deconstruction of the Cartesian Dichotomy of Black and Whitein William Blake’s The Little Black Boy Abstract This paper discusses English Romantic Poet William Blake’s anti-racial views in his poem The Little Black Boy. In so doing, it focuses upon how Blake attempts to deconstruct the Cartesian dichotomy of Western world view, a dichotomy which has usually been based on “the theory that the universe has been ruled from its origins by two conflicting powers, one good and one evil, both existing as equally ultimate first causes.” In this binary and hierarchal relationship, there are two essential terms in which one term is absolutely regarded as primary or fundamental in its essence, whereas the other term is considered secondary or something that lacks originality and presence. Once this equation is applied to the relationship between black and white people, it will easily be seen in the Western world that white people are always primary or fundamental to black-skinned people, and thus the perception behind this binary and hierarchal relationship seems the root of all the racial problems between black and white. This paper argues that Blake strives to deconstruct radically in The Little Black Boy the basis of this binary and hierarchal relationship which has been carried out for centuries in the Western world to segregate and then control the lives of black people. Finally, the paper maintains that Blake also shows a strong aspiration for creating an egalitarian society free of discrimination and injustices at a time when anti-slavery campaigns hit the top on both sides of Atlantic.

Biblionef SA: Bringing books to the bookless | Williams | Innovation

African Journals Online (AJOL)

Biblionef SA: Bringing books to the bookless. Jean Williams. Abstract. No abstract available. Innovation (2003) No. 26, pp 43-47. Full Text: EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT · AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians ...

William Knocke receives 2008 Virginia Outstanding Civil Engineer Award

OpenAIRE

Daniilidi, Christina

2008-01-01

William R. Knocke, W.C. English Professor and head of the Charles E. Via, Jr. Department of Civil and Environmental Engineering at Virginia Tech, was awarded the 2008 Virginia Outstanding Civil Engineer Award at the Virginia Section of the American Society of Civil Engineers' (ASCE) banquet, held recently in Williamsburg, Va.

Responsibility and Reliability | Williams | Philosophical Papers

African Journals Online (AJOL)

'Responsibilist\\' approaches to epistemology link knowledge and justification with epistemically responsible belief management, where responsible management is understood to involve an essential element of guidance by recognized epistemic norms. By contrast, reliabilist approaches stress the de facto reliability of ...

Pulmonary arterial stent implantation in an adult with Williams syndrome

NARCIS (Netherlands)

Reesink, Herre J.; Henneman, Onno D. F.; van Delden, Otto M.; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

2007-01-01

We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent

Variation in the Williams syndrome GTF2I gene and anxiety proneness interactively affect prefrontal cortical response to aversive stimuli.

Science.gov (United States)

Jabbi, M; Chen, Q; Turner, N; Kohn, P; White, M; Kippenhan, J S; Dickinson, D; Kolachana, B; Mattay, V; Weinberger, D R; Berman, K F

2015-08-18

Characterizing the molecular mechanisms underlying the heritability of complex behavioral traits such as human anxiety remains a challenging endeavor for behavioral neuroscience. Copy-number variation (CNV) in the general transcription factor gene, GTF2I, located in the 7q11.23 chromosomal region that is hemideleted in Williams syndrome and duplicated in the 7q11.23 duplication syndrome (Dup7), is associated with gene-dose-dependent anxiety in mouse models and in both Williams syndrome and Dup7. Because of this recent preclinical and clinical identification of a genetic influence on anxiety, we examined whether sequence variation in GTF2I, specifically the single-nucleotide polymorphism rs2527367, interacts with trait and state anxiety to collectively impact neural response to anxiety-laden social stimuli. Two hundred and sixty healthy adults completed the Tridimensional Personality Questionnaire Harm Avoidance (HA) subscale, a trait measure of anxiety proneness, and underwent functional magnetic resonance imaging (fMRI) while matching aversive (fearful or angry) facial identity. We found an interaction between GTF2I allelic variations and HA that affects brain response: in individuals homozygous for the major allele, there was no correlation between HA and whole-brain response to aversive cues, whereas in heterozygotes and individuals homozygous for the minor allele, there was a positive correlation between HA sub-scores and a selective dorsolateral prefrontal cortex (DLPFC) responsivity during the processing of aversive stimuli. These results demonstrate that sequence variation in the GTF2I gene influences the relationship between trait anxiety and brain response to aversive social cues in healthy individuals, supporting a role for this neurogenetic mechanism in anxiety.

77 FR 66087 - Federal Perkins Loan Program, Federal Family Education Loan Program, and William D. Ford Federal...

Science.gov (United States)

2012-11-01

..., and 685 Federal Perkins Loan Program, Federal Family Education Loan Program, and William D. Ford... 685 RIN 1840-AD05 [Docket ID ED-2012-OPE-0010] Federal Perkins Loan Program, Federal Family Education... (Perkins Loan) program, Federal Family Education Loan (FFEL) program, and William D. Ford Federal Direct...

Prince William Sound, Alaska ESI: ESI (Environmental Sensitivity Index Shoreline Types)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Astronaut William Fisher preparing to train in the WETF

Science.gov (United States)

1985-01-01

Astronaut William Fisher is shown in his extravehicular mobility unit (EMU) preparing to train in the Weightless Environment Training Facility (WETF). He is wearing the communications carrier assembly but not the full helmet (32102); Reflections of the WETF can be seen on the closed visor of the EMU helmet Fiser is wearing (32103).

Climatic data for Williams Lake, Hubbard County, Minnesota, 1983

Science.gov (United States)

Sturrock, A.M.; Rosenberry, D.O.; Engelbrecht, L.G.; Gothard, W.A.; Winter, T.C.

1984-01-01

Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies,including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar radiation. Data are collected at raft and land stations.

Generalized Bragg-Williams method for 'antiferromagnetic' lattice gases

International Nuclear Information System (INIS)

Osorio, R.

1983-01-01

The many-sublattice Bragg-Williams approximation of statistical mechanics is applied to the two-dimensional square and triangular lattice-gas models with nearest-neighbor repulsive interactions. Each problem is solved through both the canonical and grand-canonical methods. The present treatment emphasizes the duality between concentration and chemical potential and illustrates the appearance of first- and second -order transitions in each method. (Author) [pt

Williams Syndrome: Daily Challenges and Positive Impact on the Family

Science.gov (United States)

Scallan, Susan; Senior, Joyce; Reilly, Colin

2011-01-01

Background: Despite the distinctive physical, cognitive, personality and behavioural characteristics associated with Williams syndrome, few studies to date have examined parental experiences of raising a child with this genetic syndrome. Methods: This explorative pilot study employed predominantly qualitative methodologies via face-to-face…

77 FR 76414 - William D. Ford Federal Direct Loan Program

Science.gov (United States)

2012-12-28

... Federal Direct Loan Program AGENCY: Department of Education. ACTION: Final regulations; correction... Loan (Perkins Loan) Program; the Federal Family Education Loan (FFEL) Program; and the William D. Ford Federal Direct Loan (Direct Loan) Program, including the Public Service Loan Forgiveness (PSLF) Program...

Infrared astronomy seeing the heat : from William Herschel to the Herschel space observatory

CERN Document Server

Clements, David L

2014-01-01

Uncover the Secrets of the Universe Hidden at Wavelengths beyond Our Optical GazeWilliam Herschel's discovery of infrared light in 1800 led to the development of astronomy at wavelengths other than the optical. Infrared Astronomy - Seeing the Heat: from William Herschel to the Herschel Space Observatory explores the work in astronomy that relies on observations in the infrared. Author David L. Clements, a distinguished academic and science fiction writer, delves into how the universe works, from the planets in our own Solar System to the universe as a whole. The book first presents the major t

Finding revelation in anthropology: Alexander Winchell, William Robertson Smith and the heretical imperative.

Science.gov (United States)

Livingstone, David N

2015-09-01

Anthropological inquiry has often been considered an agent of intellectual secularization. Not least is this so in the sphere of religion, where anthropological accounts have often been taken to represent the triumph of naturalism. This metanarrative, however, fails to recognize that naturalistic explanations could sometimes be espoused for religious purposes and in defence of confessional creeds. This essay examines two late nineteenth-century figures--Alexander Winchell in the United States and William Robertson Smith in Britain--who found in anthropological analysis resources to bolster rather than undermine faith. In both cases these individuals found themselves on the receiving end of ecclesiastical censure and were dismissed from their positions at church-governed institutions. But their motivation was to vindicate divine revelation, in Winchell's case from the physical anthropology of human origins and in Smith's from the cultural anthropology of Semitic ritual.

The Role of Spiral Multidetector Dynamic CT in the Study of Williams-Campbell Syndrome

International Nuclear Information System (INIS)

Scioscio, V. di; Zompatori, M.; Mistura, I.; Montanari, P.; Santilli, L.; Luccaroni, R.; Sverzellati, N.

2006-01-01

Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results

The Role of Spiral Multidetector Dynamic CT in the Study of Williams-Campbell Syndrome

Energy Technology Data Exchange (ETDEWEB)

Scioscio, V. di; Zompatori, M.; Mistura, I.; Montanari, P.; Santilli, L.; Luccaroni, R.; Sverzellati, N. [Medical Univ. of Bologna, S. Orsola-Malpighi Policlinic (Italy). Dept. of Radiology

2006-10-15

Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results.

Climatic data for Williams Lake, Hubbard County, Minnesota, 1985

Science.gov (United States)

Sturrock, A.M.; Rosenberry, D.O.; Winter, T.C.

1987-01-01

Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies, including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Data are collected at raft and land stations.

Climatic data for Williams Lake, Hubbard County, Minnesota, 1984

Science.gov (United States)

Sturrock, A.M.; Rosenberry, D.O.; Scarborough, J.L.; Winter, T.C.

1986-01-01

Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies, including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Data are collected at raft and land stations.

Random Thoughts on William Shakespeare and Medicine

OpenAIRE

KV Sahasranam

2017-01-01

Medicine and literature have always been connected over the ages. William Shakespeare (1564-1616) is no exception. There are plenty of references to medicine and diseases in the works of Shakespeare. The knowledge which Shakespeare has of medical conditions is much more than is expected of a common man. This is attributed to his association with practitioners of his time and reading of contemporary texts in medicine. Also his son in law Dr. John Hall who married Susanna, Shakespeare's eldest ...

Mapping cultural resource sites for the Prince William Sound Graphical Resource Database

International Nuclear Information System (INIS)

Wooley, C. B.; O'Brien, D. K.; Hillman, S. O.

1997-01-01

A software package for mapping digital data 'layers' of environmentally and/or culturally sensitive areas such as seabird colonies, seal haulouts, and sea otter concentrations in Prince William Sound and adjoining areas of southern Alaska has been developed by the Alyeska Pipeline Service Company. The data is to be added to an environmental computer mapping database. More than 1,800 known and reported coastal cultural resource sites have been identified. The database is part of the Prince William Sound Tanker Oil Discharge Prevention and Contingency Plan. The mappable data layers can be used to plan and execute whatever site protection program may be necessary, thus enhancing effective cultural resource protection during an oil spill response. 22 refs., 4 figs

77 FR 32625 - William J. Stevenson, Estate of Lynn E. Stevenson, Black Canyon Bliss, LLC; Notice of Application...

Science.gov (United States)

2012-06-01

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Project No. 8866-010] William J. Stevenson, Estate of Lynn E. Stevenson, Black Canyon Bliss, LLC; Notice of Application for Transfer of License, and Soliciting Comments and Motions To Intervene On April 23, 2012, William J. Stevenson, Estate...

78 FR 33395 - Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2013-William D. Ford...

Science.gov (United States)

2013-06-04

... DEPARTMENT OF EDUCATION Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2013--William D. Ford Federal Direct Loan Program Catalog of Federal Domestic Assistance (CFDA) Number... free, at 1- 800-877-8339. SUPPLEMENTARY INFORMATION: Under the William D. Ford Federal Direct Loan...

77 FR 72960 - William D. Ford Federal Direct Loan Program

Science.gov (United States)

2012-12-07

... Federal Direct Loan Program AGENCY: Office of Postsecondary Education, Department of Education. ACTION... document to establish the date for the early implementation of William D. Ford Federal Direct Loan (Direct Loan) program regulations that establish a new income-contingent repayment plan based on the President...

William Wales and the 1769 transit of Venus: puzzle solving and the determination of the astronomical unit

Science.gov (United States)

Metz, Don

2009-05-01

According to Thomas Kuhn, a significant part of “normal science” is the fact gathering, empirical work which is intended to illustrate an existing paradigm. Some of this effort focuses on the determination of physical constants such as the astronomical unit (AU). For Kuhn, normal science is also what prepares students for membership in a particular scientific community and is embodied in some form in our science textbooks. However, neither Kuhn nor the textbook says much about the individuals who practice normal science, especially those who had been relegated to the “hack” duties of long and arduous measurement and calculation. In this paper, to provide a context for students of astronomy, I will outline the story of the determination of the AU and in particular the contribution of William Wales, an obscure British astronomer. Wales, toiling in the shadow of Halley (of Halley’s comet fame), Mason and Dixon (of Mason and Dixon line fame) and the infamous Captain Cook endured a brutal winter in northern Canada for a brief glimpse of the 1769 transit of Venus. In the end, Wales supplied one small piece of the puzzle in the determination of the AU and he exemplified the human spirit and persistence of a Kuhnian “puzzle solver”.

Case report of sudden death in a child with Williams syndrome ...

African Journals Online (AJOL)

A two year old child, confirmed with Williams syndrome (WS) ... and no relevant cardiac history such as chest pain or episodes ... brain, abdominal and pelvic organ blocks. .... a fully functional operating theatre complex presents a number.

WE-G-213-00: History Symposium: Radiological Physics Pioneers: Roentgen and the AAPM Award Eponyms - William Coolidge, Edith Quimby, and Marvin Williams - Who Were They and What Did They Do?

Energy Technology Data Exchange (ETDEWEB)

NONE

2015-06-15

Roentgen and the Birth of Modern Medical Physics – Perry Sprawls Wilhelm Roentgen is well known for his discovery of x-radiation. What is less known and appreciated is his intensive research following the discovery to determine the characteristics of the “new kind of radiation” and demonstrate its great value for medical purposes. In this presentation we will imagine ourselves in Roentgen’s mind and follow his thinking, including questions and doubts, as he designs and conducts a series of innovative experiments that provided the foundation for the rapid growth of medical physics. Learning Objectives: Become familiar with the personal characteristics and work of Prof. Roentgen that establishes him as an inspiring model for the medical physics profession. Observe the thought process and experiments that determined and demonstrated the comprehensive characteristics of x-radiation. The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who were they and what did they do? – Lawrence N. Rothenberg William David Coolidge (1873–1975) William Coolidge was born in Hudson, NY in 1873. He obtained his BS at the Massacusetts Institute of Technology in 1896. Coolidge then went to the University of Leipzig, Germany for graduate study with physicists Paul Drude and Gustave Wiedemann and received a Ph.D. in 1899. While in Germany he met Wilhelm Roentgen. Coolidge returned to the US to teach at MIT where he was associated with Arthur A. Noyes of the Chemistry Department, working on the electrical conductivity of aqueous solutions. Willis R. Whitney, under whom Coolidge had worked before going to Germany, became head of the newly formed General Electric Research Laboratory and he invited Coolidge to work with him. In 1905, Coolidge joined the staff of the GE laboratory and was associated with it for the remainder of his life. He developed ductile tungsten filaments to replace fragile carbon filaments as the material for electric light

WE-G-213-00: History Symposium: Radiological Physics Pioneers: Roentgen and the AAPM Award Eponyms - William Coolidge, Edith Quimby, and Marvin Williams - Who Were They and What Did They Do?

International Nuclear Information System (INIS)

2015-01-01

Roentgen and the Birth of Modern Medical Physics – Perry Sprawls Wilhelm Roentgen is well known for his discovery of x-radiation. What is less known and appreciated is his intensive research following the discovery to determine the characteristics of the “new kind of radiation” and demonstrate its great value for medical purposes. In this presentation we will imagine ourselves in Roentgen’s mind and follow his thinking, including questions and doubts, as he designs and conducts a series of innovative experiments that provided the foundation for the rapid growth of medical physics. Learning Objectives: Become familiar with the personal characteristics and work of Prof. Roentgen that establishes him as an inspiring model for the medical physics profession. Observe the thought process and experiments that determined and demonstrated the comprehensive characteristics of x-radiation. The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who were they and what did they do? – Lawrence N. Rothenberg William David Coolidge (1873–1975) William Coolidge was born in Hudson, NY in 1873. He obtained his BS at the Massacusetts Institute of Technology in 1896. Coolidge then went to the University of Leipzig, Germany for graduate study with physicists Paul Drude and Gustave Wiedemann and received a Ph.D. in 1899. While in Germany he met Wilhelm Roentgen. Coolidge returned to the US to teach at MIT where he was associated with Arthur A. Noyes of the Chemistry Department, working on the electrical conductivity of aqueous solutions. Willis R. Whitney, under whom Coolidge had worked before going to Germany, became head of the newly formed General Electric Research Laboratory and he invited Coolidge to work with him. In 1905, Coolidge joined the staff of the GE laboratory and was associated with it for the remainder of his life. He developed ductile tungsten filaments to replace fragile carbon filaments as the material for electric light

WEAVE: the next generation wide-field spectroscopy facility for the William Herschel Telescope : The next generation wide-field spectroscopy facility for the William Herschel Telescope

NARCIS (Netherlands)

Dalton, Gavin; Trager, Scott C.; Abrams, Don Carlos; Carter, David; Bonifacio, Piercarlo; Aguerri, J. Alfonso L.; MacIntosh, Mike; Evans, Chris; Lewis, Ian; Navarro, Ramon; Agocs, Tibor; Dee, Kevin; Rousset, Sophie; Tosh, Ian; Middleton, Kevin; Pragt, Johannes; Terrett, David; Brock, Matthew; Benn, Chris; Verheijen, Marc; Cano Infantes, Diego; Bevil, Craige; Steele, Iain; Mottram, Chris; Bates, Stuart; Gribbin, Francis J.; Rey, Jürg; Rodriguez, Luis Fernando; Delgado, Jose Miguel; Guinouard, Isabelle; Walton, Nic; Irwin, Michael J.; Jagourel, Pascal; Stuik, Remko; Gerlofsma, Gerrit; Roelfsma, Ronald; Skillen, Ian; Ridings, Andy; Balcells, Marc; Daban, Jean-Baptiste; Gouvret, Carole; Venema, Lars; Girard, Paul

We present the preliminary design of the WEAVE next generation spectroscopy facility for the William Herschel Telescope (WHT), principally targeting optical ground-based follow up of upcoming ground-based (LOFAR) and spacebased (Gaia) surveys. WEAVE is a multi-object and multi-IFU facility utilizing

Relationship of the Williams-Poulios and Manning-Rosen Potential Energy Models for Diatomic Molecules

Science.gov (United States)

Jia, Chun-Sheng; Liang, Guang-Chuan; Peng, Xiao-Long; Tang, Hong-Ming; Zhang, Lie-Hui

2014-06-01

By employing the dissociation energy and the equilibrium bond length for a diatomic molecule as explicit parameters, we generate an improved form of the Williams-Poulios potential energy model. It is found that the negative Williams-Poulios potential model is equivalent to the Manning-Rosen potential model for diatomic molecules. We observe that the Manning-Rosen potential is superior to the Morse potential in reproducing the interaction potential energy curves for the {{a}3 Σu+} state of the 6Li2 molecule and the {{X}1 sum+} state of the SiF+ molecule.

Atypical Sleep Architecture and Altered EEG Spectra in Williams Syndrome

Science.gov (United States)

Gombos, F.; Bodizs, R.; Kovacs, I.

2011-01-01

Background: Williams syndrome (WS) is a neurodevelopmental genetic disorder characterised by physical abnormalities and a distinctive cognitive profile with intellectual disabilities (IDs) and learning difficulties. Methods: In our study, nine adolescents and young adults with WS and 9 age- and sex-matched typically developing (TD) participants…

Comparison of the acute effects of Tulbaghia violacea William Henry ...

African Journals Online (AJOL)

Purpose: To assess the effect of the crude methanol leaf extracts of Tulbaghia violacea William Henry Harvey (Alliaceae) on blood pressure (BP) and heart rate in ageing normotensive Wistar Kyoto rats (WKY), and compare the results obtained with those for adult spontaneously hypertensive rats (SHR). Methods: T.

77 FR 30266 - Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2012; William D. Ford...

Science.gov (United States)

2012-05-22

... DEPARTMENT OF EDUCATION Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2012; William D. Ford Federal Direct Loan Program AGENCY: Federal Student Aid, Department of Education... announces the annual updates to the ICR plan formula for 2012. Under the William D. Ford Federal Direct Loan...

77 FR 20796 - Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2011; William D. Ford...

Science.gov (United States)

2012-04-06

... DEPARTMENT OF EDUCATION Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2011; William D. Ford Federal Direct Loan Program AGENCY: Federal Student Aid, Department of Education... announces the annual updates to the ICR plan formula for 2011. Under the William D. Ford Federal Direct Loan...

"Normality of Residuals Is a Continuous Variable, and Does Seem to Influence the Trustworthiness of Confidence Intervals: A Response to, and Appreciation of, Williams, Grajales, and Kurkiewicz (2013"

Directory of Open Access Journals (Sweden)

Jason W. Osborne

2013-09-01

Full Text Available Osborne and Waters (2002 focused on checking some of the assumptions of multiple linear.regression. In a critique of that paper, Williams, Grajales, and Kurkiewicz correctly clarify that.regression models estimated using ordinary least squares require the assumption of normally.distributed errors, but not the assumption of normally distributed response or predictor variables..They go on to discuss estimate bias and provide a helpful summary of the assumptions of multiple.regression when using ordinary least squares. While we were not as precise as we could have been.when discussing assumptions of normality, the critical issue of the 2002 paper remains -' researchers.often do not check on or report on the assumptions of their statistical methods. This response.expands on the points made by Williams, advocates a thorough examination of data prior to.reporting results, and provides an example of how incremental improvements in meeting the.assumption of normality of residuals incrementally improves the accuracy of confidence intervals.

A child of Williams-Beuren syndrome for inguinal hernia repair: Perioperative management concerns

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Sangeeta Deka

2016-01-01

Full Text Available Williams-Beuren syndrome, commonly known as Williams syndrome (WS, is a multi-organ disorder. The principal anomalies of the syndrome are developmental delay, unusual craniofacial dysmorphic features, and cardiovascular anomalies such as valvular or supravalvular aortic stenosis, pulmonary artery stenosis, and coronary insufficiency. Sudden cardiac death during minor procedures even in the absence of gross cardiovascular pathology is the most dreaded complication in these patients. A 7-year-old child with WS was posted for left-sided inguinal hernia repair under general anesthesia. Our article describes the uneventful perioperative course of the patient and highlights the concerns and complications that may be an integral part with the syndrome.

William Henry Bragg, man and scientist, Nobel Laureate and First Professor of Physics, University of Adelaide 1886-1909.

Science.gov (United States)

Patterson, John; George, Robert

2018-03-01

In London, November 1915, a telegram was received at the home of William Henry Bragg from the secretary of the Academy of Science in Stockholm announcing the award of the Nobel Prize in Physics for "the analysis of crystal structures by means of X-rays". A second similar telegram was addressed to his 25 year old son William Lawrence Bragg (Jenkin, 2008). This article commemorates the centenary of that event and the unveiling of a bust of Sir William Bragg alongside that of his son, Sir Lawrence Bragg, on North Terrace in Adelaide where he spent 23 years of his early career. Copyright © 2018. Published by Elsevier Ltd.

The Interplay between Anxiety and Social Functioning in Williams Syndrome

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Riby, Deborah M.; Hanley, Mary; Kirk, Hannah; Clark, Fiona; Little, Katie; Fleck, Ruth; Janes, Emily; Kelso, Linzi; O'Kane, Fionnuala; Cole-Fletcher, Rachel; Allday, Marianne Hvistendahl; Hocking, Darren; Cornish, Kim; Rodgers, Jacqui

2014-01-01

The developmental disorder Williams syndrome (WS) has been associated with an atypical social profile of hyper-sociability and heightened social sensitivity across the developmental spectrum. In addition, previous research suggests that both children and adults with WS have a predisposition towards anxiety. The current research aimed to explore…

Ronald William Hodges, 1934-2017: Eminent Lepidopterist and Great Mentor

Science.gov (United States)

Dr. Ronald William Hodges (1934-2017) was an eminent lepidopterist and former Research Leader of the Systematic Entomology Laboratory from 1976 to 1979. He published on 117 genera (21 new), 806 species (351 new), 36 subfamilies (7 new), and 16 families (7 new) about gelechioid micromoths. He was awa...

Inversion of the Williams syndrome region is a common polymorphism found more frequently in parents of children with Williams syndrome.

Science.gov (United States)

Hobart, Holly H; Morris, Colleen A; Mervis, Carolyn B; Pani, Ariel M; Kistler, Doris J; Rios, Cecilia M; Kimberley, Kendra W; Gregg, Ronald G; Bray-Ward, Patricia

2010-05-15

Williams syndrome (WS) is a multisystem disorder caused by deletion of about 1.55 Mb of DNA (including 26 genes) on chromosome 7q11.23, a region predisposed to recombination due to its genomic structure. Deletion of the Williams syndrome chromosome region (WSCR) occurs sporadically. To better define chance for familial recurrence and to investigate the prevalence of genomic rearrangements of the region, 257 children with WS and their parents were studied. We determined deletion size in probands by metaphase FISH, parent-of-origin of the deleted chromosome by molecular genetic methods, and inversion status of the WSCR in both parents by interphase FISH. The frequency of WSCR inversion in the transmitting parent group was 24.9%. In contrast, the rate of inversion in the non-transmitting parent group (a reasonable estimate of the rate in the general population) was 5.8%. There were no significant gender differences with respect to parent-of-origin for the deleted chromosome or the incidence of the inversion polymorphism. There was no difference in the rate of spontaneous abortion for mothers heterozygous for the WSCR inversion relative to mothers without the inversion. We calculate that for a parent heterozygous for a WSCR inversion, the chance to have a child with WS is about 1 in 1,750, in contrast to the 1 in 9,500 chance for a parent without an inversion.

Perceptual Speech and Paralinguistic Skills of Adolescents with Williams Syndrome

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Hargrove, Patricia M.; Pittelko, Stephen; Fillingane, Evan; Rustman, Emily; Lund, Bonnie

2013-01-01

The purpose of this research was to compare selected speech and paralinguistic skills of speakers with Williams syndrome (WS) and typically developing peers and to demonstrate the feasibility of providing preexisting databases to students to facilitate graduate research. In a series of three studies, conversational samples of 12 adolescents with…

How Flexible is the Use of Egocentric Versus Allocentric Frame of Reference in the Williams Syndrome Population?

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Heiz, J; Majerus, S; Barisnikov, K

2017-09-28

This study examined the spontaneous use of allocentric and egocentric frames of reference and their flexible use as a function of instructions. The computerized spatial reference task created by Heiz and Barisnikov (2015) was used. Participants had to choose a frame of reference according to three types of instructions: spontaneous, allocentric and egocentric. The performances of 16 Williams Syndrome participants between 10 and 41 years were compared to those of two control groups (chronological age and non-verbal intellectual ability). The majority of Williams Syndrome participants did not show a preference for a particular frame of reference. When explicitly inviting participants to use an allocentric frame of reference, all three groups showed an increased use of the allocentric frame of reference. At the same time, an important heterogeneity of type of frame of reference used by Williams Syndrome participants was observed. Results demonstrate that despite difficulties in the spontaneous use of allocentric and egocentric frames of reference, some Williams Syndrome participants show flexibility in the use of an allocentric frame of reference when an explicit instruction is provided. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

La haine à l’œuvre dans Carpenter’s Gothic (1999 de William Gaddis Cultivating Hatred in Carpenter’s Gothic (1999 by William Gaddis

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Jacques Sohier

2007-01-01

Full Text Available In Carpenter’s Gothic, hatred is given pride of place. William Gaddis delineates a society in which hatred proliferates. Characters are caught in the turmoils of their hatred whether it derives from the Vietnam War or from fundamentalist movements, from their individualistic quest for social recognition or from self-aggrandizement. We mean to develop a typology derived from psychoanalysis and anthropology so as to take into account all the different facets hate has in the novel. We shall devote analyses to jealousy caused by hatred, then describe the primary hate that underpins all types of relationships as a preliminary to questioning the excess that fuels Gaddis’s writing. Lastly, by referring to the author’s non-fictional work we shall put forward the thesis that the voluble protagonist is to a large extent the author’s mouthpiece. McCandless’s vindictive disquisitions on the course of the world will be seen as the equivalent of a tragic mask through which William Gaddis gives vent to his melancholia and intense detestation of the ways of the world.

William Butcher, Jules Verne inédit, les manuscrits déchiffrés

OpenAIRE

Merello, Ida

2016-01-01

Les textes de Jules Verne sont des palimpsestes. Ils le sont au sens figuré, nous le savions déjà. Mais ils le sont également au sens propre, comme le montre le bel ouvrage de William Butcher. Ce dernier s’est donné pour objectif d’analyser les manuscrits de Verne parvenus jusqu’à nous. La ressource semble inépuisable : des milliers et des milliers de pages autographes à déchiffrer, à défricher. William Butcher s’est plongé dans ce labeur avec enthousiasme et il en tire de nombreuses informat...

Analysis of the Exxon Valdez oil spill impacts on Prince William Sound pink salmon

International Nuclear Information System (INIS)

Brannon, E.L.; Moulton, L.L.; Maki, A.W.

1996-01-01

The impact of the Exxon Valdez oil spill on pink salmon in Prince William Sound (PWS), was studied. Since the incident, numerous and extensive field and laboratory studies have been completed by various scientists. This paper synthesized information on the post-spill salmon harvests, the concentration of petroleum that entered the water column, the number of streams oiled, and stream sediment chemistry, to determine the potential for oil spill effects. Results from this study showed that oiling to the extent experienced during incubation and rearing during both the spring and fall of 1989 through the spring of 1991, resulted in no measurable effects on the PWS pink salmon. The estimated losses predicted earlier, likely exceeded actual losses. 16 refs., 4 tabs., 4 figs

David Cronenberg ja William S. Burroughs kutsuvad teid lantshile / Aare Ermel

Index Scriptorium Estoniae

Ermel, Aare, 1957-2013

1999-01-01

Mängufilm "Alasti eine" ("Naked Lunch") William S. Burroughs' teose järgi : režissöör ja stsenarist David Cronenberg : Suurbritannia - Kanada 1991. Lähemalt režissööri kohta. Ka Nädal nr. 35, lk. 29

William Gaddis’s Immortality: Celebration, Cartoon, or Corruption?

OpenAIRE

Alberts, Crystal

2013-01-01

Although ostensibly a review of 'The Letters of William Gaddis' edited by Steven Moore (Champaign: Dalkey Archive, 2013), this article evaluates Moore’s volume in light of generally accepted practices of scholarly editions and the handling of historical documents. In particular, Alberts compares some of Moore’s edited letters to the originals housed either at Washington University in St. Louis or the Harry Ransom Center at the University of Texas, Austin and reveals issues involving the repre...

Musical learning in children and adults with Williams syndrome.

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Lense, M; Dykens, E

2013-09-01

There is recent interest in using music making as an empirically supported intervention for various neurodevelopmental disorders due to music's engagement of perceptual-motor mapping processes. However, little is known about music learning in populations with developmental disabilities. Williams syndrome (WS) is a neurodevelopmental genetic disorder whose characteristic auditory strengths and visual-spatial weaknesses map onto the processes used to learn to play a musical instrument. We identified correlates of novel musical instrument learning in WS by teaching 46 children and adults (7-49 years) with WS to play the Appalachian dulcimer. Obtained dulcimer skill was associated with prior musical abilities (r = 0.634, P learning strategies, but not visual or instructional strategies, predicted greater dulcimer skill beyond individual musical and visual-motor integration abilities (β = 0.285, sr(2) = 0.06, P = 0.019). These findings map onto behavioural and emerging neural evidence for greater auditory-motor mapping processes in WS. Results suggest that explicit awareness of task-specific learning approaches is important when learning a new skill. Implications for using music with populations with syndrome-specific strengths and weakness will be discussed. © 2012 The Authors. Journal of Intellectual Disability Research © 2012 John Wiley & Sons Ltd, MENCAP & IASSID.

Behavioral Profiles of Children with Williams Syndrome from Spain and the United States: Cross-Cultural Similarities and Differences

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Pérez-García, Débora; Brun-Gasca, Carme; Pérez-Jurado, Luis A.; Mervis, Carolyn B.

2017-01-01

To identify similarities and differences in the behavioral profile of children with Williams syndrome from Spain (n = 53) and the United States (n = 145), we asked parents of 6- to 14-year-olds with Williams syndrome to complete the Child Behavior Checklist 6-18. The distribution of raw scores was significantly higher for the Spanish sample than…

Religious Experience and its Essentialism in William James and Ghazzali’s Views

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Ahmad Ebadi

2016-03-01

Full Text Available Abstract Religious experience is an approach to which Western thinkers are considered pioneers among whom Schleiermacher is the most prominent. "The essentialism of religious experience" is one of the several approaches that have been adopted in the case of religious experience. Accordingly, the religion that has sides and various dimensions has been reduced to a religious experience and the religious experience is introduced as the essence of religion. What is presented in this article is a comparative study of the essence of religious experience from the perspective of William James and Ghazzāli. Although mystical experience has a different structure form the religious experience and Ghazali as well as other Muslim philosophers and mystics paid more attention to the way of mystical experience, in the works of Ghazali there are also a traces of religious experience and hence, they are adaptable to some aspects of religious experience offered by William James. William James defines the religion “as the feelings, acts, and experiences that can occur for every individual in their solitudes and he believes that feeling is the essential pillar of religion and inherently reinforces it”. Religious experience is the essence of religion and it means that-the truth of the faith is the same as feelings and emotions that emerge from rational reflections on concrete reality as such, and other spiritual, transcendental, mystical and psychological actions are the consequences of these experiences. On the other hand, in the Muslim world, Al-Ghazzali believes that: The ultimate and holy aim of religion is the perception and experience of ultimate truth that can be achieved through good deeds, worship, asceticism and piety. This article tries to find similarities and differences in essence of religious experience of the two thinkers, because it is only in the theory of the essentialism of religious experience that the similarities and differences of

William H. Sheldon and the culture of the somatotype

OpenAIRE

Gatlin, Stephen H.

1997-01-01

The burden of this dissertation is to show that William Sheldon's somatotype project should be seen as an integral aspect of modernist culture. Sheldon engaged the same problems with modernity and the "Second Industrial Revolution" (urbanization, overpopulation, industrialization, alienation) that confronted modernist poets, novelists, and philosophers. In this I am elaborating Dorothy Ross's recent metaphor, "modernist impulses in the human sciences" (1994). Both scientists and artists were ...

Unravelling starlight: William and Margaret Huggins and the rise of the new astronomy

National Research Council Canada - National Science Library

Becker, Barbara J

2011-01-01

...). A pioneer in adapting the spectroscope to new astronomical purposes, William Huggins rose to scientific prominence in London and transformed professional astronomy to become a principal founder...

Classic papers in combinatorics

CERN Document Server

Rota, Gian-Carlo

1987-01-01

This volume surveys the development of combinatorics since 1930 by presenting in chronological order the fundamental results of the subject proved in over five decades of original papers by:.-T. van Aardenne-Ehrenfest.-R.L. Brooks.-N.G. de Bruijn.-G.F. Clements.-H.H. Crapo.-R.P. Dilworth.-J. Edmonds.-P.Erdös.-L.R. Ford, Jr.-D.R. Fulkerson.-D. Gale.-L. Geissinger.-I.J. Good.-R.L. Graham.-A.W. Hales.-P. Hall.-P.R. Halmos.-R.I. Jewett.-I. Kaplansky.-P.W. Kasteleyn.-G. Katona.-D.J. Kleitman.-K. Leeb.-B. Lindström.-L. Lovász.-D. Lubell.-C. St. J.A. Nash-Williams.-G. Pólya.-F.P. Ramsey.-G.C. Rota.-B.L. Rothschild.-H.J. Ryser.-C. Schensted.-M.P. Schützenberger.-R.P. Stanley.-G. Szekeres.-W.T. Tutte.-H.E. Vaughan.-H. Whitney.

Anterior segment dysgenesis associated with Williams-Beuren syndrome: a case report and review of the literature.

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Todorova, Margarita G; Grieshaber, Matthias C; Cámara, Rafael J A; Miny, Peter; Palmowski-Wolfe, Anja M

2014-05-21

Williams-Beuren syndrome is characterized by mild mental retardation, specific neurocognitive profile, hypercalcemia during infancy, distinctive facial features and cardiovascular diseases. We report on complete ophthalmologic, sonographic and genetic evaluation of a girl with a clinical phenotype of Williams-Beuren syndrome, associated with unilateral anterior segment dysgenesis and bilateral cleft of the soft and hard palate. These phenotypic features have not been linked to the haploinsufficiency of genes involved in the microdeletion. A term born girl presented at the initial examination with clouding of the right cornea. On ultrasound biomicroscopy the anterior chamber structures were difficult to differentiate, showing severe adhesions from the opacified cornea to the iris with a kerato-irido-lenticular contact to the remnant lens, a finding consistent with Peters' anomaly. Genetic analyses including FISH confirmed a loss of the critical region 7q11.23, usually associated with the typical Williams-Beuren syndrome. Microsatellite analysis showed a loss of about 2.36 Mb. A diagnosis of Williams-Beuren syndrome was made based on the microdeletion of 7q11.23. The unique features, including unilateral microphthalmia and anterior segment dysgenesis, were unlikely to be caused by the microdeletion. Arguments in favor of the latter are unilateral manifestation, as well as the fact that numerous patients with deletions of comparable or microscopically visible size have not shown similar manifestations.

Epigenesis and the rationality of nature in William Harvey and Margaret Cavendish.

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Goldberg, Benjamin

2017-06-01

The generation of animals was a difficult phenomenon to explain in the seventeenth century, having long been a problem in natural philosophy, theology, and medicine. In this paper, I explore how generation, understood as epigenesis, was directly related to an idea of rational nature. I examine epigenesis-the idea that the embryo was constructed part-by-part, over time-in the work of two seemingly dissimilar English philosophers: William Harvey, an eclectic Aristotelian, and Margaret Cavendish, a radical materialist. I chart the ways that they understood and explained epigenesis, given their differences in philosophy and ontology. I argue for the importance of ideas of harmony and order in structuring their accounts of generation as a rational process. I link their experiences during the English Civil war to how they see nature as a possible source for the rationality and concord sorely missing in human affairs.

Application of progressive nucleation mechanism for the citation behavior of individual papers of different authors.

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Sangwal, Keshra

2012-09-01

The basic concepts and equations of the progressive nucleation mechanism (PNM) are presented first for the growth and decay of items. The mechanism is then applied to describe the cumulative citations L and citations ΔL per year of the individual most-cited papers i of four selected Polish professors as a function of citation duration t. It was found that the PNM satisfactorily describes the time dependence of cumulative citations L of the papers published by different authors with sufficiently high citations ΔL, as represented by the highest yearly citations ΔL(max) during the entire citation period t (normal citation behavior). The citation period for these papers is less than 15 years and it is even 6-8 years in several cases. However, for papers with citation periods exceeding about 15 years, the growth behavior of citations does not follow the PNM in the entire citation period (anomalous citation behavior), and there are regions of citations in which the citation data may be described by the PNM. Normal and anomalous citation behaviors are attributed, respectively, to the occurrence and nonoccurrence of stationary nucleation of citations for the papers. The PNM also explains the growth and decay of citations ΔL per year of papers exhibiting normal citation behavior.

Categories are alive: interview with Brackette F. Williams

OpenAIRE

Durão, Susana; Bastos, Cristiana; Williams, Brackette F.

2013-01-01

Brackette F. Williams is an American anthropologist and a Senior Justice ­Advocate, currently an associate professor of Cultural Anthropology at the University of ­Arizona. She studied at the University of Cornell, the University of Arizona, and received her doctorate from Johns Hopkins University. Her work interweaves ­matters of race, gender, class, ideology and politics in a unique manner – as seen in the article “A class act: Anthropology and the race to nation across ethnic terrain” (Ann...

Fissured and geographic tongue in Williams-Beuren syndrome

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Neeta Sharma

2014-01-01

Full Text Available Williams-Beuren Syndrome (WBS is a rare, most often sporadic, genetic disease caused by a chromosomal microdeletion at locus 7q11.23 involving 28 genes. It is characterized by congenital heart defects, neonatal hypercalcemia, skeletal and renal abnormalities, cognitive disorder, social personality disorder, and dysmorphic facies. A number of clinical findings has been reported, but none of the studies evaluated this syndrome considering oral cavity. We here report a fissured and geographic tongue in association with WBS.

Leftward lateralization of auditory cortex underlies holistic sound perception in Williams syndrome.

Science.gov (United States)

Wengenroth, Martina; Blatow, Maria; Bendszus, Martin; Schneider, Peter

2010-08-23

Individuals with the rare genetic disorder Williams-Beuren syndrome (WS) are known for their characteristic auditory phenotype including strong affinity to music and sounds. In this work we attempted to pinpoint a neural substrate for the characteristic musicality in WS individuals by studying the structure-function relationship of their auditory cortex. Since WS subjects had only minor musical training due to psychomotor constraints we hypothesized that any changes compared to the control group would reflect the contribution of genetic factors to auditory processing and musicality. Using psychoacoustics, magnetoencephalography and magnetic resonance imaging, we show that WS individuals exhibit extreme and almost exclusive holistic sound perception, which stands in marked contrast to the even distribution of this trait in the general population. Functionally, this was reflected by increased amplitudes of left auditory evoked fields. On the structural level, volume of the left auditory cortex was 2.2-fold increased in WS subjects as compared to control subjects. Equivalent volumes of the auditory cortex have been previously reported for professional musicians. There has been an ongoing debate in the neuroscience community as to whether increased gray matter of the auditory cortex in musicians is attributable to the amount of training or innate disposition. In this study musical education of WS subjects was negligible and control subjects were carefully matched for this parameter. Therefore our results not only unravel the neural substrate for this particular auditory phenotype, but in addition propose WS as a unique genetic model for training-independent auditory system properties.

Leftward lateralization of auditory cortex underlies holistic sound perception in Williams syndrome.

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Martina Wengenroth

Full Text Available BACKGROUND: Individuals with the rare genetic disorder Williams-Beuren syndrome (WS are known for their characteristic auditory phenotype including strong affinity to music and sounds. In this work we attempted to pinpoint a neural substrate for the characteristic musicality in WS individuals by studying the structure-function relationship of their auditory cortex. Since WS subjects had only minor musical training due to psychomotor constraints we hypothesized that any changes compared to the control group would reflect the contribution of genetic factors to auditory processing and musicality. METHODOLOGY/PRINCIPAL FINDINGS: Using psychoacoustics, magnetoencephalography and magnetic resonance imaging, we show that WS individuals exhibit extreme and almost exclusive holistic sound perception, which stands in marked contrast to the even distribution of this trait in the general population. Functionally, this was reflected by increased amplitudes of left auditory evoked fields. On the structural level, volume of the left auditory cortex was 2.2-fold increased in WS subjects as compared to control subjects. Equivalent volumes of the auditory cortex have been previously reported for professional musicians. CONCLUSIONS/SIGNIFICANCE: There has been an ongoing debate in the neuroscience community as to whether increased gray matter of the auditory cortex in musicians is attributable to the amount of training or innate disposition. In this study musical education of WS subjects was negligible and control subjects were carefully matched for this parameter. Therefore our results not only unravel the neural substrate for this particular auditory phenotype, but in addition propose WS as a unique genetic model for training-independent auditory system properties.

Adaptive Behavior and Problem Behavior in Young Children with Williams Syndrome

Science.gov (United States)

Hahn, Laura J.; Fidler, Deborah J.; Hepburn, Susan L.

2014-01-01

The present study compares the adaptive behavior profile of 18 young children with Williams syndrome (WS) and a developmentally matched group of 19 children with developmental disabilities and examines the relationship between adaptive behavior and problem behaviors in WS. Parents completed the Vineland Adaptive Behavioral Scales--Interview…

40 CFR Appendix B to Part 425 - Modified Monier-Williams Method

Science.gov (United States)

2010-07-01

... 40 Protection of Environment 29 2010-07-01 2010-07-01 false Modified Monier-Williams Method B Appendix B to Part 425 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) EFFLUENT GUIDELINES AND STANDARDS LEATHER TANNING AND FINISHING POINT SOURCE CATEGORY Pt. 425, App. B Appendix B to...

I Know! It's Backwards Day! Gender Roles and William's Doll

Science.gov (United States)

Van de Kleut, Geraldine

2007-01-01

This article presents a case study of an exploration of gender roles in a second-grade classroom. The author discusses some of the discursive identities in which she and her students are positioned, and then uses the picture book William's Doll to introduce a discussion of discursive gender identities with her students. She then asks students to…

Elicited Production of Relative Clauses in Children with Williams Syndrome

Science.gov (United States)

Zukowski, Andrea

2009-01-01

Relative clauses have been implicated alternately as a strength and a weakness in the language of people with Williams Syndrome (WS). To clarify the facts, an elicited production test was administered to 10 people with WS (age 10-16 years), 10 typically developing children (age 4-7 years), and 12 typically developing adults. Nearly every WS…

TENNESSEE WILLIAMS E O TEATRO MARGINAL GAY

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Adriana Falqueto Lemos

2014-06-01

Full Text Available The work developed in this text aims to read the dramatist Tennnessee Williams in a play in two scenes “E Contar Tristes Histórias das Mortes das Bonecas” which was published in Brazil in the book “Mister Paradise e outras peças em um ato” (2011. The intention is to reflect upon one of his recurring themes, the marginalization. In order to perform the analysis, the theoretical support was grounded in “Literatura e Sociedade” by Antonio Candido (2006, concerning the participation of society and authorship in a piece of literature.

TENNESSEE WILLIAMS E O TEATRO MARGINAL GAY

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Adriana Falqueto Lemos

2014-09-01

Full Text Available The work developed in this text aims to read the dramatist Tennnessee Williams in a play in two scenes “E Contar Tristes Histórias das Mortes das Bonecas” which was published in Brazil in the book “Mister Paradise e outras peças em um ato” (2011. The intention is to reflect upon one of his recurring themes, the marginalization. In order to perform the analysis, the theoretical support was grounded in “Literatura e Sociedade” by Antonio Candido (2006, concerning the participation of society and authorship in a piece of literature.

77 FR 17568 - Notice of Receipt of Petition for Decision That Nonconforming 2005 Ifor Williams LM85G Trailers...

Science.gov (United States)

2012-03-26

...-0030, Notice 1] Notice of Receipt of Petition for Decision That Nonconforming 2005 Ifor Williams LM85G... Safety Administration (NHTSA) of a petition for a decision that 2005 Ifor Williams LM85G trailers that... capable of being altered to comply with, all applicable FMVSS based on destructive test data or such other...

Afinidades seletivas: uma comparação entre as sociobiologias da literatura de Pierre Bourdieu e Raymond Williams Selective kinships: comparing the literarture sociologies od Pierre Bourdieu and Raymond Williams

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Enio Passiani

2009-10-01

Full Text Available As sociologias da literatura de Bourdieu e Williams estão apoiadas em dois conceitos fundamentais: “habitus” e “estruturas de sentimento”, respectivamente. E é clara a semelhança entre eles: ambos tentam traduzir uma espécie de consciência prática adquirida pelos agentes sociais a partir de um processo particular de socialização. Para o primeiro, o “habitus” é internalizado na experiência vivida num campo específi co; ao passo que, para o segundo, tal consciência é formada no interior dos grupos culturais. Nesse sentido, ambas as noções dizem respeito a um conjunto de valores compartilhados que aproximam e afastam certos agentes uns dos outros, criando solidariedade e rivalidades. Esta comunicação pretende demonstrar que, apesar das diferenças que existem, os conceitos dos autores não supervalorizam nem a reprodução nem a mudança social, mas, ao contrário, tentam explicar esses fenômenos como jogo dialético. Palavras-chave: Pierre Bourdieu. Raymond Williams. Sociologia da literatura. “Habitus”. Estruturas de sentimento. The literature sociologies of Bourdieu and Williams are structured in two fundamental concepts: “habitus” and “sentiment structures”, respectively. It’s very clear the resemblance between them: both try to translate a kind of practical conscience, acquired by the social agents from a particular socializing process. To the fi rst, the “habitus” is internalized in the living experience of a specifi c fi eld; while that to the second that conscience is formed in the interior of cultural groups. In this sense, both ideas talk about shared values which approximate and withdraw some agents one from the others, creating solidarity and competition. This text intends to show that, despite of the existing differences, the authors concepts don’t overestimate neither the reproduction, nor the social change, but, otherwise, try to explain these phenomena as a dialectic game. Keywords

Developments in fiber-positioning technology for the WEAVE instrument at the William Herschel Telescope

Science.gov (United States)

Schallig, Ellen; Lewis, Ian J.; Gilbert, James; Dalton, Gavin; Brock, Matthew; Abrams, Don Carlos; Middleton, Kevin; Aguerri, J. Alfonso L.; Bonifacio, Piercarlo; Carrasco, Esperanza; Trager, Scott C.; Vallenari, Antonella

2016-08-01

WEAVE is the next-generation wide-field optical spectroscopy facility for the William Herschel Telescope (WHT) on La Palma in the Canary Islands, Spain. It is a multi-object "pick-and-place" fibre-fed spectrograph with a 1000 fibre multiplex behind a new dedicated 2° prime focus corrector. The WEAVE positioner concept uses two robots working in tandem in order to reconfigure a fully populated field within the expected 1 hour dwell-time for the instrument (a good match between the required exposure times and the limit of validity for a given configuration due to the effects of differential refraction). In this paper we describe some of the final design decisions arising from the prototyping phase of the instrument design and provide an update on the current manufacturing status of the fibre positioner system.

Sir William Osler's speech at Troy: a Trojan horse?

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Moran, Michael E

2012-01-01

Troy, New York, is a city of 55,000 people in upstate New York located along the Hudson River. A city of surprisingly rich cultural heritage, it was the home of New York state's first hospital outside New York City. The 50th anniversary celebration of Troy's hospital brought William Osler to the city as the keynote speaker. This speech, delivered on November 28, 1900, is one of Sir William's less well known addresses. Osler began his comments with Sir Thomas More's Utopia and talked at length about the hospital, its obligations, the influences it has upon the community, and the role of physicians and surgeons. He broached one of his old saws, the salary of attending physicians and their needed role in hospital management. His words were published in the diamond jubilee's records, but the hospital did not outlive its prominent guest professor, and it closed its doors in 1914. Just like the great historical city of Troy, New York's own Troy was on the brink of decline, and its hospital would be the first fatality. Therefore, it is almost prescient that the words of Osler, taken into historical context juxtaposed against the socioeconomic forces at work, are akin to the Greek's offering of a wooden edifice to end the Trojan War.

Meteor Beliefs Project: some meteoric imagery in the works of William Shakespeare

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McBeath, A.; Gheorghe, A. D.

2003-08-01

Passages from three of William Shakespeare's plays are presented, illustrating some of the beliefs in meteors in 16th-17th century England. They also reflect earlier beliefs and information which it is known Shakespeare drew on in constructing his works.

Síndrome de Williams-Beuren. Enfoque diagnóstico a través del fenotipo

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María Angelina Lacruz-Rengel

2015-10-01

Full Text Available Resumen (español El síndrome de Williams-Beuren es un trastorno genético del desarrollo neurológico que incluye diferentes manifestaciones clínicas como retardo en el crecimiento e intelectual, cardiopatía congénita y rasgo facial distintivo. El objetivo de este estudio es describir las características clínicas que conducen a su diagnóstico, conocer su evolución fenotípica y las medidas terapéuticas adecuadas. Se realizó un estudio retrospectivo, descriptivo, tipo serie de casos, en el que se incluyeron todos los pacientes con el diagnóstico de síndrome de Williams-Beuren, evaluados en el Instituto Autónomo Hospital Universitario de Los Andes, durante enero de 2000 a junio de 2014. El principal motivo de consulta fue la presencia de dismorfía facial y cardiopatía congénita, en la totalidad de los casos. La estenosis de la arteria pulmonar bilateral fue el tipo de cardiopatía congénita más frecuente en 4/7 pacientes. El síndrome de Williams-Beuren es una entidad genética que muestra un amplio espectro de características clínicas. Conocer todos estos hallazgos permite realizar el diagnóstico, ofrecer una adecuada atención médica, psicoterapéutica y educativa que permite mejorar su calidad de vida, sobrevida e inserción a la sociedad Abstract (english Williams-Beuren syndrome is a genetic neurodevelopmental disorder that includes different clinical manifestations as growth retardation and intellectual, congenital heart disease and distinctive facial features. The aim of this study was to describe the clinical features that leading to their diagnosis, know their phenotypic evolution and provide appropriate treatment. A retrospective, descriptive case series study, which included all patients with a diagnosis of Williams-Beuren syndrome, evaluated in the Autonomous Institute University Hospital of Los Andes, from January 2000 to June 2014 was performed. The main complaint was the presence of facial dysmorphic and

Shoreline oiling conditions in Prince William Sound following the Exxon Valdez oil spill

International Nuclear Information System (INIS)

Neff, J.M.; Owens, E.H.; Stoker, S.W.; McCormick, D.M.

1995-01-01

Following the Exxon Valdez oil spill of March 24, 1989, in Prince William Sound, Alaska, Exxon conducted comprehensive, systematic shoreline surveys in cooperation with federal and state authorities to obtain information on the distribution and magnitude of shoreline oiling and to identify natural and cultural resources requiring special protection. Similar joint surveys were performed during the springs of 1990, 1991, and 1992 on all Prince william Sound and Gulf of Alaska shorelines that were suspected of having remnants of weathered oil and that would benefit from further cleanup. In the springs of 1990, 1991, and 1992, isolated pockets of subsurface oil were found, chiefly in small scattered zones in coarse cobble/boulder sediments in the upper intertidal or supratidal zones. In 1991, about one-third of the subdivisions in Prince William Sound with surface oil also contained some subsurface oil. The areal extent of this subsurface oil declined by nearly 70% between 1991 and 1992, from about 37,000 m 2 to about 12,000 m 2 . Moreover, where subsurface oil remained in 1992, it was present in lesser amounts. Rates of oil removal were greatest on coastal sections treated early in the spring and summer of 1989. Where shoreline treatment was delayed, the subsequent rate of removal of oil from the shore by natural processes was slower. 27 refs., 10 figs., 3 tabs

La Fondation William et Flora Hewlett | CRDI - Centre de recherches ...

International Development Research Centre (IDRC) Digital Library (Canada)

La Fondation William et Flora Hewlett. http://www.hewlett.org/. Initiative Think tank. Cette initiative permet d'établir des organismes indépendants qui se consacrent à la recherche et à l'élaboration de politiques dans des pays en développement. Voir davantage. Initiative Think tank. Croissance de l'économie et débouchés ...

Case report of sudden death in a child with Williams syndrome ...

African Journals Online (AJOL)

Williams syndrome is a neurodevelopmental disorder characterized by distinctive personality traits, facial features (so called “elfin face”) and cardiac abnormalities, of which supravalvular aortic stenosis is the most common lesion found. The cause is a deletion of a group of genes on chromosome 7q11.23. Administration of ...

William Golding's Iconoclastic Views about the Neanderthal Man in "The Inheritors"

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Youssef, Sayed Mohammed

2016-01-01

William Golding has been identified as a nonconformist whose opinions always go contrary to what is customarily accepted or established. This is shown in all his novels, more specifically "The Inheritors", in which he defies long established opinions held by anthropologists, historians, archaeologists as well as many others about the…

Physiology or psychic powers? William Carpenter and the debate over spiritualism in Victorian Britain.

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Delorme, Shannon

2014-12-01

This paper analyses the attitude of the British Physiologist William Benjamin Carpenter (1813-1885) to spiritualist claims and other alleged psychical phenomena in the second half of the Nineteenth Century. It argues that existing portraits of Carpenter as a critic of psychical studies need to be refined so as to include his curiosity about certain 'unexplained phenomena', as well as broadened so as to take into account his overarching epistemological approach in a context of theological and social fluidity within nineteenth-century British Unitarianism. Carpenter's hostility towards spiritualism has been well documented, but his interest in the possibility of thought-transference or his secret fascination with the medium Henry Slade have not been mentioned until now. This paper therefore highlights Carpenter's ambivalences and focuses on his conciliatory attitude towards a number of heterodoxies while suggesting that his Unitarian faith offers the keys to understanding his unflinching rationalism, his belief in the enduring power of mind, and his effort to resolve dualisms. Copyright © 2014 Elsevier Ltd. All rights reserved.

Climatic data for Williams Lake, Hubbard County, Minnesota, 1982

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Rosenberry, D.O.; Sturrock, A.M.; Scarborough, J.L.; Winter, T.C.

1988-01-01

Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Those climatic data needed for energy budget and mass transfer studies are presented , including: water surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Some calculated values necessary for these studies are also presented, such as vapor pressure and Bowen-ratio values. Data are collected at raft and land stations.

Climatic data for Williams Lake, Hubbard County, Minnesota, 1986

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Rosenberry, D.O.; Sturrock, A.M.; Winter, T.C.

1988-01-01

Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies, including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Some calculated values necessary for these studies, such as vapor pressure and Bowen ratio numbers, also are presented. Data are collected at raft and land stations.

Centennial ties: Harvey Cushing (1869-1939) and William Osler (1849-1919) on Andreas Vesalius (1514-1564).

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Toodayan, Nadeem

2017-08-01

Andreas Vesalius is often regarded as the founding father of modern anatomical study. The quincentennial anniversary of his birth - 31 December 2014 - has been very widely commemorated, and it is the purpose of this article to contrast these celebrations with what happened during the Vesalius quatercentenary year of 1914. More specifically, we look at how Vesalius was perceived a century ago by examining his influence on two of western medicine's most iconic gentlemen - Harvey Williams Cushing (1869-1939) and his larger than life mentor, Sir William Osler (1849-1919).

William Gowers: the never completed third edition of the 'Bible of Neurology'.

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Eadie, Mervyn J; Scott, Ann E M; Lees, Andrew J; Woodward, Martin

2012-10-01

William Gowers' classic single-authored two-volume A manual of diseases of the nervous system appeared in a first edition in 1886 and 1888, and in a second edition in 1892 and 1893, with a third edition of Volume 1 in 1899. No third edition of Volume 2 ever appeared. However, in 1949 Critchley stated that he had seen part of a revision of this volume. Subsequent writers could not find this material, but it recently came to light at Gowers' old hospital at Queen Square, London. The present paper describes the rediscovered material, containing Gowers' handwritten alterations for a further edition of Volume 2, and substantial new material, at least in relation to nystagmus and myasthenia. Gowers' declining health, or a conflict between his planned new text and his contributions to the neurology segments (1899) of Allbutt's System of medicine, may explain why a third edition of Volume 2 of the Manual of diseases of the nervous system never appeared.

Simulasi Perdagangan Saham PT Tambang Batubara Bukit Asam, Tbk. dengan Macd dan Williams % Range

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Benny Benny

2010-11-01

Full Text Available Moving Average Convergence Divergence (MACD is a momentum indicator to determine the indication of the trend reversal point. Williams% Range is also a momentum indicator that can provide when reversal trend will occur. This analysis will use the data movement of stock prices for PT Tambang Coal Bukit Asam Tbk. (PTBA. PTBA stock prices are influenced by fundamental conditions such as limited supply of fuel which is rumored and coal is the main alternative fuels other than oil. Study period was 241 trade days. Data are analyzed in several periods and then fitted with the MACD indicator and Williams % R, then simulate the trade during the period of research studies to determine the level of trading profits or losses based on the combination of both these technical indicators. The conclusion of this analysis, the MACD is an indicator that is considered slow (lagging in giving an indication of weakness, but in reverse the MACD is considered to provide an accurate indication of where the indication given is rarely wrong. Williams% R is an rapid indicator in anticipation of a reversal trend, but these indicators often give a false indication since this indicator is quite sensitive to the movement of stock prices goings go up or down. 

Incontinence in children, adolescents and adults with Williams syndrome.

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von Gontard, Alexander; Niemczyk, Justine; Borggrefe-Moussavian, Sorina; Wagner, Catharina; Curfs, Leopold; Equit, Monika

2016-11-01

Williams Syndrome (WS) is a microdeletion syndrome (chromosome 7q11.23) characterized by typical facial features, cardiovascular disease, behavioural symptoms, and mild intellectual disability (ID). The aim of this study was to assess the rates of incontinence and psychological problems in persons with WS. 231 individuals with WS were recruited through the German parent support group (52.0% male, mean age 19.4 years). Faecal incontinence (FI) was diagnosed from the age of 4 years and nocturnal enuresis (NE) and daytime urinary incontinence (DUI) of 5 years onwards. The Parental Questionnaire: Enuresis/Urinary Incontinence, the International-Consultation-on-Incontinence-Questionnaire-Pediatric LUTS (ICIQ-CLUTS), as well as the Developmental Behavior Checklist for parents (DBC-P) or for adults (DBC-A) were filled out by parents or caregivers. 17.8% of the sample had NE, 5.9% DUI and 7.6% FI. NE was present in 44.9% of children (4-12 years), 13.5% of teens (13-17y), 3.3% of young adults (18-30y) and in 3.6% of adults (> 30y). DUI (and FI) decreased from 17.9% (21.4%) in children to 0% in adults. 3.5% of the sample had an ICIQ-CLUTS score in the clinical range. 30.5% of children and 22.1% of adults had a clinical DBC score. Children and teens with clinically relevant DBC-P-scores had significantly higher DUI rates. Children with WS have high rates of incontinence and LUTS, which decrease with age. Most adults are continent. NE is the most common subtype. Except for DUI in children, incontinence is not associated with behavioural problems. Screening, assessment and treatment of incontinence in individuals with WS is recommended. Neurourol. Urodynam. 35:1000-1005, 2016. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.

Validação do escore ultra-sonográfico de Williams para o diagnóstico da hepatopatia da fibrose cística Validation of the Williams ultrasound scoring system for the diagnosis of liver disease in cystic fibrosis

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Eleonora D. T. Fagundes

2004-01-01

Full Text Available OBJETIVOS: Descrever as alterações hepáticas observadas ao exame ultra-sonográfico de fibrocísticos do Ambulatório de Fibrose Cística do Hospital das Clínicas da UFMG, comparar os achados ultra-sonográficos com critérios clínicos e bioquímicos e validar o escore de Williams para o diagnóstico de hepatopatia associada à fibrose cística. MÉTODOS: Setenta fibrocísticos foram acompanhados prospectivamente e submetidos a exame clínico, bioquímico e ultra-sonográfico. Os achados ultra-sonográficos foram comparados com os resultados do exame clínico e bioquímico. Para a validação do escore ultra-sonográfico de Williams, os critérios clínicos e bioquímicos foram utilizados como padrão-ouro. Foram calculados sensibilidade, especificidade, valor preditivo positivo e valor preditivo negativo do escore de Williams. Para os cálculos, os pacientes foram divididos em dois grupos: normal ao exame ultra-sonográfico (escore = 3 ou alterado (escore > 3. RESULTADOS: Dez pacientes preencheram os critérios clínicos e/ou bioquímicos para hepatopatia (14,3%. Todos os pacientes hepatopatas segundo os critérios clínicos e/ou bioquímicos apresentavam alguma alteração ao exame ultra-sonográfico. As alterações do parênquima hepático, borda hepática e fibrose periportal foram encontradas mais freqüentemente entre os hepatopatas, com diferença estatisticamente significativa. O escore de Williams apresentou alta especificidade (91,7%; IC 80,9-96,9, mas baixa sensibilidade (s = 50%; IC 20,1-79,9 para o diagnóstico da hepatopatia. CONCLUSÕES: O escore de Williams não constituiu um bom exame de triagem quando comparado ao exame clínico e bioquímico. Uma vez que ainda não há nenhum teste que, utilizado isoladamente, apresente sensibilidade adequada, é recomendável a utilização conjunta dos exames clínico, bioquímico e ultra-sonográfico no diagnóstico da hepatopatia associada à fibrose cística, sempre em avalia

The Recurring Author: William Shakespeare, a Case Study through Content Analysis.

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Harrison, Robert L., Jr.

The "recurring author" is one whose works appear many times at different levels in instructional units found in literature textbook series. A descriptive case study discussed the treatment of a recurring author, William Shakespeare, using units in a sample of six literature textbook series. Developed to describe, to code, and to analyze…

Prosodic Abilities of Spanish-Speaking Adolescents and Adults with Williams Syndrome

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Martinez-Castilla, Pastora; Sotillo, Maria; Campos, Ruth

2011-01-01

In spite of the relevant role of prosody in communication, and in contrast with other linguistic components, there is paucity of research in this field for Williams syndrome (WS). Therefore, this study performed a systematic assessment of prosodic abilities in WS. The Spanish version of the Profiling Elements of Prosody in Speech-Communication…

Diurnal Cortisol Profile in Williams Syndrome in Novel and Familiar Settings

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Lense, Miriam Diane; Tomarken, Andrew J.; Dykens, Elisabeth M.

2013-01-01

Williams syndrome (WS) is a neurodevelopmental genetic disorder associated with high rates of anxiety and social issues. We examined diurnal cortisol, a biomarker of the stress response, in adults with WS in novel and familiar settings, and compared these profiles to typically developing (TD) adults. WS and TD participants had similar profiles in…

William Gaddis’s Immortality: Celebration, Cartoon, or Corruption?

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Crystal Alberts

2013-11-01

Full Text Available Although ostensibly a review of 'The Letters of William Gaddis' edited by Steven Moore (Champaign: Dalkey Archive, 2013, this article evaluates Moore’s volume in light of generally accepted practices of scholarly editions and the handling of historical documents. In particular, Alberts compares some of Moore’s edited letters to the originals housed either at Washington University in St. Louis or the Harry Ransom Center at the University of Texas, Austin and reveals issues involving the representation of the archival material, inconsistencies in editorial principles, and a misattributed letter, among other things.

Social-emotional and behavioral adjustment in children with Williams-Beuren syndrome.

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Gosch, A; Pankau, R

1994-12-01

In children with Williams-Beuren syndrome (WBS), disturbed behaviors (neurotic, antisocial, and hyperactive) [Arnold et al., 1985: Dev Med Child Neurol 27:49-59; Udwin et al., 1987: J Child Psychol Psychiat 28:297-309] have been described. To study the behavior disturbances and social-emotional adjustment in children with WBS, a group of N = 19 patients was compared with a control group, matched for age, gender, and nonverbal reasoning abilities. Parents were asked to assess the children's behavior in terms of a list of 20 items of the Child Behavior Checklist (CBCL) [Achenbach and Edelbrock, 1983: Manual for the Child Behavior Checklist] and the Vineland Social Maturity Scale (VSMS) [Lüer et al., 1972: Kurzform der Vineland Social Maturity Scale]. As compared with the control group, children with WBS differ significantly in their social behavior towards strangers. They exhibit no reserve or distancing behavior and would, for instance, follow a stranger without hesitation. They are described as showing a hypersensitivity to sounds that is more pronounced than in the control group. Finally, they are found to be significantly less well-adjusted socially than the control individuals.

WILLIAM HAZLITT, OBSESSIVE LOVE, AND LIBER AMORIS.

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Trosman, Harry

2017-07-01

William Hazlitt, a distinguished literary figure of the early nineteenth century and a forerunner of psychoanalytic insights, had a keen awareness of the impact of the imagination on assessing works of art. At forty-two, he became hopelessly involved in an obsessive love affair with a nineteen-year-old woman and could not extricate himself from the relationship. The affair followed the death of his father, a powerful influence on his life. Factors in his obsessive love included finding an object of idealization subject to his imaginative creation and narcissistically reexperiencing himself about to begin a new life. © 2017 The Psychoanalytic Quarterly, Inc.

Social organization of sea otters in Prince William Sound, Alaska

Science.gov (United States)

Garshelis, David L.; Johnson, Ancel M.; Garshelis, Judith A.

1984-01-01

Sea otters in Prince William Sound. Alaska, were spatially segregated into predominantly (97%) male areas at the front of the expanding population and breeding areas with fewer (up to 33%) males. From 1975 to 1984 we captured and marked 267 otters with tags and (or) radio transmitters and investigated their reproductive strategies, social relationships, and patterns of sexual segregation. Mating occurred year-round, but peaked in September and October. Females first bred at 4 years of age and were capable of pupping annually; they generally separated from their pup before mating. Males established breeding territories that enabled prolonged precopulatory interactions that may have prompted female–pup separation and post-copulatory interactions that precluded females from mating with other males. Male mating success was related to age, weight, territory quality, and the length of time they maintained their territory. After the breeding season, territorial males returned to male areas where food was more abundant. Young, dispersing males also entered male areas and remained there until attaining breeding age. In male areas, otters commonly rested in groups of >50 individuals. Gregariousness promoted social interactions and likely enhanced food finding and (historically) predator protection. As food diminished, males moved into adjacent, unoccupied regions; females then occupied former male areas.

Featural versus configural face processing in a rare genetic disorder: Williams syndrome

NARCIS (Netherlands)

Isaac, L.; Lincoln, A.

2011-01-01

Background Williams syndrome (WMS) is a rare genetic disorder with an estimated prevalence of 1 in 20 000 live births. Among other characteristics, WMS has a distinctive cognitive profile with spared face processing and language skills that contrasts with impairment in the cognitive domains of

John Whitridge Williams, MD (1866–1931) of Baltimore: pioneer of academic obstetrics

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Dunn, P M

2007-01-01

Williams was the founder of academic obstetrics in the United States and with his textbook was the recognised leader of this discipline in America during the first 30 years of the 20th century. PMID:17185435

William J. Donovan: Visionary, Strategic Leader, an Historical Perspective

Science.gov (United States)

1991-05-03

War II years until his death . 1945 through 1959. Strategy, without Information on which It can rely, is helpless. Information Is useless unless It Is...was again emulating his Rough Rider hero, because like him, he was now a cavalryman.32 Donovan had never been a horseman or soldier before, but like...Intelligence officer and Chief of Combined Operatlrns during World War II, said of General William Donovan In 1966, seven years after his death , "This

OBITUARY: Sir William Mitchell Physicist and enthusiast for science

CERN Multimedia

Cowley, R

2002-01-01

"William Mitchell was successively head of Physics at Reading and Oxford universities, and Chairman of the Science and Engineering Research Council from 1985 to 1990. He is largely responsible for the excellent neutron and X-ray facilities that are available for research by scientists in the UK. He was one of the first to realise that these uniquely powerful facilities would be essential tools not only for physicists but also for chemists, biologists, materials scientists and engineers" (1 page).

Structural Analysis of the Oxymoron in the Sonnets of William Shakespeare

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Liliya R. Sakaeva

2017-11-01

Full Text Available This paper considers the structural groups of oxymoron in the Russian and English languages. It is also relevant to study oppositional lexical units represented in heterogeneous system languages from the standpoint of linguistic and extra linguistic meanings, since the figures of contrast are inconceivable without the associative-emotional and evaluative qualifications of the objects of opposition. They give the analysis of the oxymoron’s nature and its functions in two different-structured languages. The article has carried out lexical and semantic characteristics of oxymoron. In the linguistic literature there is no generalized, concrete and universal structural and semantic classification of this stylistic device. This study attempts to create a structural and semantic classification, combining all the existing varieties of this figure of contrast. The analysis is applied in the linguistic examination of the Sonnets written by William Shakespeare. When studying, systemizing and analyzing the opposite units, it is extremely important to study their structural features. The main objective of this study is to identify and describe the types of oxymoron in the language of Shakespeare’s sonnets.

Sleep EEG Fingerprints Reveal Accelerated Thalamocortical Oscillatory Dynamics in Williams Syndrome

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Bodizs, Robert; Gombos, Ferenc; Kovacs, Ilona

2012-01-01

Sleep EEG alterations are emerging features of several developmental disabilities, but detailed quantitative EEG data on the sleep phenotype of patients with Williams syndrome (WS, 7q11.23 microdeletion) is still lacking. Based on laboratory (Study I) and home sleep records (Study II) here we report WS-related features of the patterns of…

Pedagogical progeniture or tactical translation? George Fordyce's additions and modifications to William Cullen's philosophical chemistry--Part II.

Science.gov (United States)

Taylor, Georgette

2014-08-01

This paper compares the affinity theories and the associated affinity diagrams of William Cullen (1710-1790) and George Fordyce (1736-1802), exploring in particular one episode that took place during the brief hiatus between Fordyce's student years at Edinburgh University and the start of his own pedagogical career in London. This investigation complements that contained in Part I of this paper, which compared the chemistry courses given by Cullen and Fordyce, demonstrating that the knowledge originally imparted to Fordyce by Cullen in his Edinburgh lectures was augmented and translated by Fordyce for his own pedagogical purposes. Part II offers greater insight into the flow of knowledge between Fordyce and Cullen. Their correspondence suggests that the relationship between master and student transmuted into something more complicated after Fordyce left Edinburgh, while the model of knowledge transmission between the two can be seen to be more collaborative than might be expected.

Williams syndrome and its cognitive profile: the importance of eye movements

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Van Herwegen J

2015-06-01

Full Text Available Jo Van Herwegen Department of Psychology, Kingston University London, Surrey, UK Abstract: People with Williams syndrome (WS, a rare neurodevelopmental disorder that is caused by a deletion on the long arm of chromosome 7, often show an uneven cognitive profile with participants performing better on language and face recognition tasks, in contrast to visuospatial and number tasks. Recent studies have shown that this specific cognitive profile in WS is a result of atypical developmental processes that interact with and affect brain development from infancy onward. Using examples from language, face processing, number, and visuospatial studies, this review evaluates current evidence from eye-tracking and developmental studies and argues that domain general processes, such as the ability to plan or execute saccades, influence the development of these domain-specific outcomes. Although more research on eye movements in WS is required, the importance of eye movements for cognitive development suggests a possible intervention pathway to improve cognitive abilities in this population. Keywords: Williams syndrome, eye movements, face processing, language, number, visuospatial abilities

Williams Syndrome and Anesthesia for Non-cardiac Surgery: High Risk Can Be Mitigated with Appropriate Planning.

Science.gov (United States)

Brown, Morgan L; Nasr, Viviane G; Toohey, Rebecca; DiNardo, James A

2018-03-23

Patients with Williams syndrome are considered at high risk for anesthesia-related adverse events. At our institution, all William syndrome patients undergoing cardiac surgical, cardiac catheterization/interventional procedures, and cardiac imaging studies are cared for by cardiac anesthesiologists. All William syndrome patients undergoing non-cardiac surgical, interventional, or imaging studies are cared for by main operating room pediatric anesthesiologists with consultative input from a cardiac anesthesiologist. We reviewed our experience with 75 patients undergoing 202 separate anesthetics for 95 non-cardiac procedures and 107 cardiac procedures from 2012 to 2016. The mean age was 7.5 ± 7.0 years and the mean weight was 22.3 ± 17.0 kg. One hundred and eighty-seven patients had a general anesthetic (92.6%). Medications used included etomidate in 26.2%, propofol in 37.6%, isoflurane in 47.5%, and sevoflurane in 68.3%. Vasopressors and inotropes were required including calcium (22.8%), dopamine (10.4%), norepinephrine (17.3%), phenylephrine (35.1%), vasopressin (0.5%), and ephedrine (5.4%). The median length of stay after anesthesia was 2.8 days (range 0-32). No adverse events occurred in 89.6% of anesthetics. There were two cases of cardiac arrest, one of which required extracorporeal life support for resuscitation. Of the non-cardiac surgical procedures, 95.7% did not have a cardiovascular adverse event. Patients with Williams syndrome are at high risk for anesthesia, especially when undergoing cardiac procedures. The risk can be mitigated with appropriate planning and adherence to the hemodynamic goals for non-cardiac surgical procedures.

William Harvey & Capillaries: Can You "Discover" Circulation without Connections between Arteries & Veins?

Science.gov (United States)

Allchin, Douglas

2005-01-01

William Harvey's discovery of the presence of capillaries and the role played by them without actually seeing them highlights his power of observation and logic. However, his perspective could not be considered as new since he had adopted his disposition for ocular demonstration from Aristotle and Galen.

Drug-Coated Balloon Angioplasty: A Novel Treatment for Pulmonary Artery In-Stent Stenosis in a Patient with Williams Syndrome.

Science.gov (United States)

Cohen, Jennifer L; Glickstein, Julie S; Crystal, Matthew A

2017-12-01

A 20-month-old boy with Williams syndrome had undergone multiple surgical and catheter-based interventions for resistant peripheral pulmonary arterial stenoses with eventual bilateral stent placement and conventional balloon angioplasty. He persistently developed suprasystemic right ventricular (RV) pressure. Angioplasty with a drug-coated balloon (DCB) was performed for in-stent restenosis and to remodel his distal pulmonary vessels bilaterally. This resulted in immediate improvement in the in-stent stenosis and resultant decrease in RV pressure. Follow-up catheterization two months later continued to show long-lasting improvement in the in-stent stenosis. We hypothesize that the anti-proliferative effects of DCBs may be of benefit in the arteriopathy associated with Williams syndrome. We report this as a novel use of a DCB in the pulmonary arterial circulation in a patient with Williams syndrome.

Lähtugem ehitistest! / William J. R. Curtis ; interv. Andres Kurg, Karin Hallas, Triin Ojari

Index Scriptorium Estoniae

Curtis, William J. R.

1998-01-01

24. apr. pidas Rotermanni soolalaos loengu arhitektuuriajaloolane William J. R. Curtis. Katkeid jutuajamisest W. Curtisega. Arhitektuurikriitikast, oma raamatust "Modern Architecture since 1900", millest W. Curtis praegu kriitikuna kirjutab, Lille'i projektist jm

Parents perceptions of social inclusion for children with Williams Syndrome

OpenAIRE

Gulliver, Katherine

2016-01-01

This thesis explores parents’ perceptions of social inclusion for children with Williams Syndrome; a rare intellectual disability with a distinct social cognitive profile. 5 interviews with parents give rich understanding to what parents’ value for their child’s education, and how this is achieved. Thematic analysis highlights key similarities and differences in experiences between parents of children attending mainstream schools and one special school. Severity of disability affects how pare...

Morphological differences in the mirror neuron system in Williams Syndrome

OpenAIRE

Ng, Rowena; Brown, Timothy T.; Erhart, Matthew; Järvinen, Anna M.; Korenberg, Julie R.; Bellugi, Ursula; Halgren, Eric

2015-01-01

Williams syndrome (WS) is a genetic condition characterized by an overly gregarious personality, including high empathetic concern for others. Although seemingly disparate from the profile of autism spectrum disorder (ASD), both are associated with deficits in social communication/cognition. Notably, the mirror neuron system (MNS) has been implicated in social dysfunction for ASD; yet, the integrity of this network and its association with social functioning in WS remains unknown. Magnetic re...

The Impact of Personality on History: An Interview with William L. Shirer.

Science.gov (United States)

Social Education, 1983

1983-01-01

William L. Shirer, author of Rise and Fall of the Third Reich, discusses a wide variety of subjects, including the personalities of Roosevelt and Hitler, why the Germans succumbed to Nazism, McCarthyism in the United States, and the heroic resistance of the Russians to German invasion. (CS)

The Production of Figurative Language in Typically Developing Children and Williams Syndrome

Science.gov (United States)

Naylor, Lauren; Van Herwegen, Jo

2012-01-01

The current study investigated the development of figurative language production, including different types of figurative expressions, during a fictional narrative in 20 typically developing (TD) children and 20 children with Williams syndrome (WS) aged 7-18 years old. In contrast to previous studies, developmental trajectories showed that (1) the…

Random Thoughts on William Shakespeare and Medicine

Directory of Open Access Journals (Sweden)

KV Sahasranam

2017-04-01

Full Text Available Medicine and literature have always been connected over the ages. William Shakespeare (1564-1616 is no exception. There are plenty of references to medicine and diseases in the works of Shakespeare. The knowledge which Shakespeare has of medical conditions is much more than is expected of a common man. This is attributed to his association with practitioners of his time and reading of contemporary texts in medicine. Also his son in law Dr. John Hall who married Susanna, Shakespeare's eldest daughter would have contributed substantially to the knowledge of medicine in Shakespeare's compositions. Surgery at the Elizabethan times was well known and is reflected to a large extent in his plays.

Selfish memes: An update of Richard Dawkins’ bibliometric analysis of key papers in sociobiology

OpenAIRE

Aaen-Stockdale, Craig

2017-01-01

This is an Open Access journal available from http://www.mdpi.com/ In the second edition of The Selfish Gene, Richard Dawkins included a short bibliometric analysis of key papers instrumental to the sociobiological revolution, the intention of which was to support his proposal that ideas spread within a population in an epidemiological manner. In his analysis, Dawkins primarily discussed the influence of an article by British evolutionary biologist William Donald Hamilton which had introdu...

Emotion Recognition as a Real Strength in Williams Syndrome: Evidence From a Dynamic Non-verbal Task

Directory of Open Access Journals (Sweden)

Laure Ibernon

2018-04-01

Full Text Available The hypersocial profile characterizing individuals with Williams syndrome (WS, and particularly their attraction to human faces and their desire to form relationships with other people, could favor the development of their emotion recognition capacities. This study seeks to better understand the development of emotion recognition capacities in WS. The ability to recognize six emotions was assessed in 15 participants with WS. Their performance was compared to that of 15 participants with Down syndrome (DS and 15 typically developing (TD children of the same non-verbal developmental age, as assessed with Raven’s Colored Progressive Matrices (RCPM; Raven et al., 1998. The analysis of the three groups’ results revealed that the participants with WS performed better than the participants with DS and also than the TD children. Individuals with WS performed at a similar level to TD participants in terms of recognizing different types of emotions. The study of development trajectories confirmed that the participants with WS presented the same development profile as the TD participants. These results seem to indicate that the recognition of emotional facial expressions constitutes a real strength in people with WS.

Patterns and Trajectories in Williams Syndrome: The Case of Visual Orientation Discrimination

Science.gov (United States)

Palomares, Melanie; Englund, Julia A.; Ahlers, Stephanie

2011-01-01

Williams Syndrome (WS) is a developmental disorder typified by deficits in visuospatial cognition. To understand the nature of this deficit, we characterized how people with WS perceive visual orientation, a fundamental ability related to object identification. We compared WS participants to typically developing children (3-6 years of age) and…

Factor Structure of the Children's Behavior Questionnaire in Children with Williams Syndrome

Science.gov (United States)

Leyfer, Ovsanna; John, Angela E.; Woodruff-Borden, Janet; Mervis, Carolyn B.

2012-01-01

To examine the factor structure of temperament in 5-10-year-olds with Williams syndrome, an exploratory factor analysis was conducted on the responses of parents of 192 children on the children's behavior questionnaire. Four factors were identified. Two corresponded to factors reported for typically developing children: effortful control and…

Time–frequency analysis of nonstationary complex magneto-hydro-dynamics in fusion plasma signals using the Choi–Williams distribution

International Nuclear Information System (INIS)

Xu, L.Q.; Hu, L.Q.; Chen, K.Y.; Li, E.Z.

2013-01-01

Highlights: • Choi–Williams distribution yields excellent time–frequency resolution for discrete signal. • CWD method provides clear time–frequency pictures of EAST and HT-7 fast MHD events. • CWD method has advantages to wavelets transform scalogram and the short-time Fourier transform spectrogram. • We discuss about how to choose the windows and free parameter of CWD method. -- Abstract: The Choi–Williams distribution is applied to the time–frequency analysis of signals describing rapid magneto-hydro-dynamic (MHD) modes and events in tokamak plasmas. A comparison is made with Soft X-ray (SXR) signals as well as Mirnov signal that shows the advantages of the Choi–Williams distribution over both continuous wavelets transform scalogram and the short-time Fourier transform spectrogram. Examples of MHD activities in HT-7 and EAST tokamak are shown, namely the onset of coupling tearing modes, high frequency precursors of sawtooth, and low frequency MHD instabilities in edge localized mode (ELM) free in H mode discharge

Tracing sediment sources in the Williams River catchment using caesium-137 and heavy metals: towards an assessment of the relative importance of surface erosion and gully erosion

International Nuclear Information System (INIS)

Krause, A.K.; Kalma, J.D.; Loughran, R.J.

1999-01-01

Recent sediment sourcing in the 1175km 2 Williams River catchment near Newcastle, NSW, has involved the use of caesium-137 ( 137 Cs) and heavy metals to identify zones of erosion and estimate erosion rates. Sediment sources to the Williams River include sheet erosion from forested and grazed lands, stream channels (especially banks), gullies and roads. The fallout environmental radioisotope 137 Cs was used to assess the erosion status of five vegetated slopes using soil sampling along transects. The net loss or gain of 137 Cs at each sampling point was compared with the 137 Cs level at a reference site at the slope crest. Net soil loss at each point was calculated from an Australian regression model relating net soil loss from runoff-erosion plots to 137 Cs deficit in soils (n=34; r=0.84). Net soil gain was calculated using the regression model in reverse mode. A weighted net soil loss (or gain) was then calculated for each slope transect. Results showed low net soil loss, ranging from zero to 0.64 t ha -1 yr 1 , suggesting that slopes were not major contributors of sediment to the Williams River. A small sub-catchment south of Wirragulla Hill, typical of the lower Williams region, was selected for more detailed tracing of sediment sources. The catchment contains gullies, sheet-erosion exposed sub-soil, grassland and one unsealed road. Heavy metals and 137 Cs have been used to fingerprint the sources, and these measurements will be compared with suspended sediment collected from drainage water in the creek. Only preliminary results have been obtained for this component of the study. The paper will assess these two approaches for the identification of sediment sources and discuss practical applications in water resources management

Williams Sendromu Olan Bireylerin Ailelerinin Deneyimlerinin ve Karşılaştıkları Güçlüklerin Belirlenmesi

Directory of Open Access Journals (Sweden)

Deniz Tekin Ersan

2016-12-01

Full Text Available Williams Sendromu, belirli bir kromozomda silinme meydana gelmesi ile oluşan nörogelişimsel bir bozukluktur. Görülme sıklığı düşük olan Williams Sendromu ile ilgili yapılmış çalışmalar hem ülkemizde hem de dünyada son derece sınırlıdır. Bu araştırma ile Williams Sendromu olan bireylerin ailelerinin yaşadığı deneyimlerin ve güçlüklerin belirlenmesi amaçlanmaktadır. Araştırmanın modeli nitel araştırma yöntemidir. Araştırmaya beş Williams Sendromu olan bireyin ailesi katılmıştır. Araştırmada veriler, yarı yapılandırılmış görüşme yöntemi ile ve telefon aracılığı ile toplanmıştır. Veriler betimsel analiz ile analiz edilmiştir. Araştırma bulgularına göre; katılımcıların tümünün Williams Sendromu olan bir çocuğa sahip olmayı zor bir deneyim olarak değerlendirdikleri, çocuklarının geleceğine yönelik kaygı taşıdıkları gözlenmiştir. Ailelerin yaşadıkları güçlüklere bakıldığında özellikle çocuklarının davranış problemlerine dair yaşanan güçlükler öne çıkmaktadır. Bunun yanında eğitim ve sağlık alanında uzmanların konu ile ilgili bilgisinin yetersizliği, sahip oldukları olumsuz tutumlar ve aynı zamanda verilen hizmetlerin yetersizliği ile ilgili görüşler öne çıkmaktadır. Ek olarak, kaynakların azlığı ve bilgiye ulaşmanın zorluğuna ilişkin bulgular da elde edilmiştir. Williams Syndrome is a neuro-developmental disorder, which is caused by a deletion in a certain chromosome. Prevalence of Williams Syndrome is low and there is limited number of studies on Williams Syndrome both in our country and abroad. The purpose of this study is to determine experiences and difficulties of parents who have children with Williams Syndrome. It is a qualitative study. Five parents of children with Williams Syndrome were participated in the study. Data collected through semi structured interview via phone calls. Data were analyzed

Williams-Beuren syndrome: pitfalls for diagnosis in limited resources setting

OpenAIRE

Lumaka, Aimé; Lukoo, Rita; Mubungu, Gerrye; Lumbala, Paul; Mbayabo, Gloire; Mupuala, Aimée; Lukusa-Tshilobo, Prosper; Devriendt, Koenraad

2016-01-01

Key Clinical Message Patients with Williams?Beuren Syndrome can be recognized clinically, given the characteristic dysmorphism, intellectual disability, and behavior. We report on a Congolese boy with typical WBS facial characteristics. He suffered meningitis and coma at the age of 2 years then subsequently presented with profound intellectual disability and atypical behavior. The WBS was only made at age 8.2 years and confirmed with FISH testing and microarray?CGH. The present report aims to...

William H. Taft High School Project HOLA, 1985-1986. OEA Evaluation Report.

Science.gov (United States)

New York City Board of Education, Brooklyn. Office of Educational Assessment.

Project HOLA at William H. Taft High School (Bronx, New York) assists foreign-born and Puerto Rican-born students to quickly assists foreign- and Puerto Rican-born students to quickly acquire English language skills and an American cultural orientation; to maintain or improve their Spanish language skills and cultural knowledge; and to be…

William H. Taft High School Project HOLA, 1986-1987. OEA Evaluation Report.

Science.gov (United States)

Martinez, Ana L.; And Others

In its fourth year of Title VII funding, Project HOLA at William H. Taft High School served 383 Spanish-speaking students of limited English proficiency from low-income families. The program's goals were to develop English language skills for mainstreaming, to develop an understanding and awareness of American culture and society, to develop…

William Bagley versus Arthur Bestor: Why the Standard Story Is Not True

Science.gov (United States)

Null, J. Wesley

2008-01-01

This essay challenges the conventional understanding of William Bagley and Arthur Bestor, which suggests that they held similar views in curriculum and teacher education. The author thinks this view is completely wrong and provides a radical new interpretation of Bagley and Bestor that uncovers a lost tradition within the field of education.…

Parent and Teacher Perspectives about Problem Behavior in Children with Williams Syndrome

Science.gov (United States)

Klein-Tasman, Bonita P.; Lira, Ernesto N.; Li-Barber, Kirsten T.; Gallo, Frank J.; Brei, Natalie G.

2015-01-01

Problem behavior of 52 children with Williams syndrome ages 6 to 17 years old was examined based on both parent and teacher report. Generally good inter-rater agreement was found. Common areas of problem behavior based both on parent and teacher report included attention problems, anxiety difficulties, repetitive behaviors (e.g., obsessions,…

The Use of Cohesive Markers in Narratives by Children with Williams Syndrome

Science.gov (United States)

Jones, Nancy Elizabeth

2013-01-01

This study examined how children and adolescents with Williams syndrome (WS; ages 8 years, 0 months [8;0]-14;5) used referential devices (determiners and pronouns), tense, and connectives to create cohesion in oral narratives based on a storybook compared to typically developing mentally and chronologically age-matched children. WS children used…

‘Wot larx!’: William Morris, Charles Dickens, and Fatherly Feelings

Directory of Open Access Journals (Sweden)

Wendy Parkins

2011-09-01

Full Text Available This essay explores the significance of William Morris’s reading of Dickens for Morris’s articulation of fatherly feelings. Recent scholarship on Victorian fatherhood has begun to overturn the stereotype of the dour, emotionally distant paterfamilias as providing only a partial understanding of forms of middle-class fatherhood and William Morris may serve as an example of a Victorian father whose parenting style eschewed detached authoritarianism, and instead combined nurture, play, and creativity. While Morris referred to Dickens’s works and characters in letters to a range of correspondents, his repeated usage of Joe Gargery’s catchphrase ‘Wot larx!’ (variously spelled occurs exclusively in letters to his wife and daughters, especially the latter. I will consider how the character of Joe Gargery, who combines nurturing tenderness with manly labour, was deployed by Morris to perform a playful and affectionate paternal persona. While Morris’s use of Joe’s catchphrase seems to express a spontaneous overflow of powerful feeling its status as a quotation also works to mark (and mask a disavowal of powerfully ambivalent feelings concerning his own emotional agency as husband and father.

Pre-spill shoreline mapping in Prince William Sound, Alaska

International Nuclear Information System (INIS)

Owens, E.H.; Lamarche, A.; Reimer, P.D.; Marchant, S.O.; O'Brien, D.K.

2003-01-01

A long-term shoreline mapping program has been initiated in Prince William Sound, Alaska, to generate pre-spill data to assist in the planning activities for oil spill response in the area. Low-altitude aerial videotape surveys and video images form the basis for the mapping effort. The coast was initially divided into alongshore segments. The physical shore-zone is relatively homogeneous within each segment. A pre-spill Shoreline Cleanup Assessment Team (SCAT) database, using the ShoreData software, was created based on this initial detailed mapping. The SCAT field teams are therefore equipped with a detailed analysis of the shore-zone character. The same information was also used to develop a separate database for use by planning and response operations groups. The data is entered into the Graphical Resource Database (GRD), and a Geographic Information System (GIS). A simplified characterization of the primary features of each segment is then made available through interpretation of the data. In the event of an oil spill, the SCAT data in the ShoreData files can be combined with field data on shoreline oiling conditions using a second software package called ShoreAccess R which provides summaries of the main parameters required by the planning group. It can also be used as a data storage and management tool. As part of this program, more than 1700 kilometres of shoreline in Prince William Sound have already been mapped. 24 refs., 4 tabs., 5 figs

'…to whom it will be extremly Usefull.' Dr William Cullen's adoption of James Watt's copying machine.

Science.gov (United States)

Wheelock, H

2016-06-01

Dr William Cullen (1710-1790) was a leading physician of the Enlightenment era. As professor in Edinburgh he became the most influential teacher of theoretical and practical medicine in 18th century Britain. A renowned private practitioner, Cullen systematically archived his postal 'consultations', now held by the Royal College of Physicians of Edinburgh. Initially Cullen preserved his replies as transcriptions, but from April 1781 he began using a mechanical copier, newly devised by the Scottish engineer James Watt. This paper describes the development, promotion and functioning of Watt's copier and considers Cullen's own adoption of the machine. It is suggested that with Cullen's adoption of Watt's copier, medical record keeping entered a new historical phase comparable with the recent digital revolution.

Dr William Farr of Shropshire (1807-1883): obstetric mortality and training.

Science.gov (United States)

Dunn, P M

2002-07-01

William Farr, chief statistician to the General Register Office for more than 40 years, was the most significant medical epidemiologist and statistician of the Victorian era. Often working behind the scenes, he helped to bring about many advances in hygiene and public health as well as developing a modern approach to the classification of disease and the collection and analysis of medical information data.

Aortic anomalies in an adolescent with the Williams' elfin facies syndrome

International Nuclear Information System (INIS)

Williams, R.L.; Azouz, E.M.

1984-01-01

An adolescent with classical Williams' syndrome who presented with hypertensive encephalopathy is described. He had the unusual combination of supravalvular aortic stenosis, long segment coarctation of the aorta, aortic hypoplasia and a high bifurcation of the abdominal aorta. Surgical resection of the coarctation was required; however, the patient has remained moderately hypertensive. The aortic anomalies in this syndrome are reviewed and their frequency and importance examined. (orig.)

Nothing personal : algorithmic individuation on music streaming platforms

NARCIS (Netherlands)

Prey, Robert

2017-01-01

Raymond Williams once wrote, ‘… there are in fact no masses, but only ways of seeing people as masses’. In an age of personalized media, the word ‘masses’ seems like an anachronism. Nevertheless, if Williams were to study contemporary online platforms, he would no doubt conclude that there are in

Ramparts of empire the fortifications of sir william jervois, royal engineer 1821 - 1897

CERN Document Server

Crick, Timothy

2015-01-01

William Jervois was a military engineer who rose to prominence as a result of Lord Palmerston's extensive programme of fortification against a feared French invasion in the middle years of the nineteenth century. Ramparts of Empire is a detailed and engaging study of his life and works.

Cortisol Reactivity and Performance Abilities in Social Situations in Adults with Williams Syndrome

Science.gov (United States)

Lense, Miriam D.; Dykens, Elisabeth M.

2013-01-01

Williams syndrome (WS) is a neurodevelopmental disorder associated with hypersociability and anxiety. However, little is known about how these salient aspects of the phenotype are related or their underlying physiology. We examined cortisol reactivity in WS because cortisol is responsive to psychosocial stress. Compared to typically developing…

Sir William Mitchell (1925-2002)

CERN Multimedia

2002-01-01

Sir William (Bill) Mitchell, former President of the CERN Council, died on 30th October 2002 at the age of 77. Mitchell was professor of Physics at Oxford University from 1978 to 1989, having previously been Professor of Physics, Dean of Science and Deputy Vice-Chancellor at Reading University. From 1985 to 1990 he was Chairman of the UK's Science and Engineering Research Council (SERC), which at the time was the funding agency for the UK's participation in the CERN programme. As Chairman, Mitchell was one of the UK representatives on the CERN Council, and in 1991 he was elected President, a position he held for three years. This was a difficult period for CERN. Financial problems were being faced in many member states, notably in Germany as a result of unification. This led to calls for reductions in the CERN budget and, more significantly, to requests for delays in consderation of future programmes. On the other hand for the future of CERN and the progress of elementary particle physics, it was necessary...

David Owen Williams (1944 - 2006)

CERN Multimedia

2006-01-01

Many people, not only at CERN but also throughout the world, were saddened to learn that their friend and colleague David Williams had passed away in the early hours of Tuesday 24 October. His death came after a year of fighting cancer with all of his usual determination and optimism. Even days before the end he was still welcoming to visitors, and was alert and interested in all their news. Born in 1944, David came to CERN from the University of Cambridge in 1966, with a degree in Physics and Computer Science. Joining what at the time was called the Documents and Data (DD) Division, in the earlier part his career he worked first on software for analysis of bubble chamber photographs, subsequently leading the group that supported experiments with 'hybrids' of bubble chambers and electronic detectors and then the group supporting online computing in experiments. He thus witnessed all of the enormous changes that took place in particle physics as the era of bubble chambers came to an end and the availability ...

William L. Donn 1918-1987

Science.gov (United States)

Gedzelman, Stanley David

William L. Donn, Professor Emeritus of the Department of Earth and Planetary Sciences, City College of New York, and Special Research Scientist at Lamont-Doherty Geological Observatory (LDGO) of Columbia University (Palisades, N.Y.), died at his home on June 30, 1987, at the age of 69. Bill demonstrated expertise in a wide range of fields, with a highly productive and creative research and writing career that included geology, oceanography, climatology, atmospheric physics, and meteorology.Donn was born in Brooklyn, N.Y., on March 2, 1918. At the tender age of 10 years, he demonstrated his love and talent for science by building a telescope with his brother, Bertram. During his undergraduate years at Brooklyn College, he switched his major from astronomy to geology. He was largely selftrained in both meteorology and oceanography, serving as head of the Meteorology Section, U.S. Merchant Marine Academy during World War II . One by-product of these years was the textbook Meteorology—With Marine Applications, first published in 1946. This widely adopted text became a standard for a generation of mariners and college students.

Item and Error Analysis on Raven's Coloured Progressive Matrices in Williams Syndrome

Science.gov (United States)

Van Herwegen, Jo; Farran, Emily; Annaz, Dagmara

2011-01-01

Raven's Coloured Progressive Matrices (RCPM) is a standardised test that is commonly used to obtain a non-verbal reasoning score for children. As the RCPM involves the matching of a target to a pattern it is also considered to be a visuo-spatial perception task. RCPM is therefore frequently used in studies in Williams Syndrome (WS), in order to…

[William W. Cadbury and canton hospital].

Science.gov (United States)

Liu, Ze-Sheng; Liu, Ze-En

2004-01-01

William W. Cadbury M.D. was born in Philadelphia, USA and graduated from the Medical College of Pennsylvania University. It was nearly 40 years since he arrived in Canton (Guangzhou) in 1909 and left at retirement age. He taught western medicine in Canton Christian College and worked as a medical doctor in Canton Hospital, the oldest western medical hospital in the Orient. He was regarded as a famous foreign doctor and an excellent professor in internal medicine in the Republic of China. He wrote At the point of Lancet: 100 years of Canton Hospital 1835 - 1935, which recorded the achievement made by American missionary doctors, particularly the pioneers such as Peter Parker M.D. and John G. Kerr. M.D. So far the book is still an important reference for the studies on history of western medicine in China and the history of modern medical exchange between China and other countries.

Evaluation of sea otter capture after the Exxon Valdez oil spill, Prince William Sound, Alaska

Science.gov (United States)

Bodkin, James L.; Weltz, F.; Bayha, Keith; Kormendy, Jennifer

1990-01-01

After the T/V Exxon Valdez oil spill into Prince William Sound, the U.S. Fish and Wildlife Service and Exxon Company, U.S.A., began rescuing sea otters (Enhydra lutris). The primary objective of this operation was to capture live, oiled sea otters for cleaning and rehabilitation. Between 30 March and 29 May 1989, 139 live sea otters were captured in the sound and transported to rehabilitation centers in Valdez, Alaska. Within the first 15 days of capture operations, 122 (88%) otters were captured. Most sea otters were captured near Knight, Green, and Evans islands in the western sound. The primary capture method consisted of dipnetting otters out of water and off beaches. While capture rates declined over time, survival of captured otters increased as the interval from spill date to capture date increased. The relative degree of oiling observed for each otter captured declined over time. Declining capture rates led to the use of tangle nets. The evidence suggests the greatest threat to sea otters in Prince William Sound occurred within the first 3 weeks after the spill. Thus, in the future, the authors believe rescue efforts should begin as soon as possible after an oil spill in sea otter habitat. Further, preemptive capture and relocation of sea otters in Prince William Sound may have increased the number of otters that could have survived this event.

Charles William Lacaillade. Biologist, Parasitologist, Educator, and Mentor.

Science.gov (United States)

Imperato, Pascal James

2017-02-01

Charles William Lacaillade (1904-1978) was an eminent biologist in the middle decades of the twentieth century. He was born in Lawrence, Massachusetts of parents whose ancestors were French Canadians. His father, also named Charles William Lacaillade, was a dentist who graduated from Tufts University School of Dentistry in 1898. His mother, Elodia Eno, came from a family of very successful businessmen. Lacaillade was the third of six children. His two older brothers, Harold Carleton and Hector Eno, both graduated from the University of Louisville, School of Dentistry, while his younger brother, Lawrence, became a businessman. His sister, Luemma, married Dr. Henry Steadman, a veterinarian, while his youngest sister, Gloria, married a U.S. Army officer, Lieutenant Colonel Victor Anido. Lacaillade received his MS and PhD degrees in biology and zoology from Harvard University. He then became a fellow at The Rockefeller Institute for Medical Research. At both institutions, he studied under some of the most eminent biological scientists of the time. These included Rudolf W. Glaser, George Howard Parker, Theobald Smith, Carl TenBroeck, and William Morton Wheeler. At the Rockefeller Institute, he co-discovered the vector and mode of transmission of Eastern Equine Encephalomyelitis. This discovery, and the research he conducted with Rudolf W. Glaser, quickly established him as an outstanding biological researcher. However, a change in leadership at the Rockefeller Institute resulted in research priorities being given to the disciplines of general physiology, physical chemistry, and nutrition. This shift in the research agenda away from the biological sciences precluded career advancement at the Rockefeller Institute for post-doctoral fellows like Lacaillade. It was the height of the Great Depression, and even biologists with terminal doctoral degrees found it difficult to find positions. In 1935, Lacaillade accepted a position as an assistant in biology at St. John

Obituary: William K. Rose (1935-2010)

Science.gov (United States)

Trimble, Virginia

2011-12-01

Stellar astrophysicist William Kenneth Rose died near his home in Potomac, Maryland, on September 30, 2010, after an extended illness. Rose was the son of pharmacist Kenneth William Rose and Shirley Near Rose and was born in Ossining, New York, on August 10, 1935. He received an AB from Columbia College in 1957 and a PhD in physics from Columbia University in 1963, with a thesis on "measurements of linear polarization in discrete radio sources using a 9.4 cm maser," under the direction of Charles H. Townes. Rose played a major role in designing and constructing the maser and used it at a radio telescope at Maryland Point that belonged to the Naval Research Lab. He observed Jupiter and Saturn and a number of extra-solar-system sources, and also diffuse centimeter emission (see appendix). The thesis was not published in an archival journal, but can be found under Library of Congress code QB 475.R67. While in graduate School, Bill married Sheila Tuchman, whose primary scientific interests were biological. None of their three children chose to be scientists, but two are CPAs. Bill moved successfully through the academic hurdles) from a research position at Princeton (1963-67), where a collaboration with Nick Woolf and Martin Schwarzchild on the infrared spectra of giant stars became one of his most-cited papers, to assistant and associate professorships at MIT (1967-71), and then associate and full professorships at the University of Maryland (1971 to retirement in 2005). His most innovative work was probably that on nova explosions arising from degenerate ignition of hydrogen accreted on white dwarfs in close binary systems, published in 1968. The same idea occurred to others at about the same time, and Bill did not, perhaps, get quite his fair share of the credit. I first met Sheila and Bill in summer 1969 at the Stony Brook summer school on stellar evolution (not published until 1972). He lectured on the nature of nova explosions and on nuclear burning in thin

(A)musicality in Williams syndrome: examining relationships among auditory perception, musical skill, and emotional responsiveness to music.

Science.gov (United States)

Lense, Miriam D; Shivers, Carolyn M; Dykens, Elisabeth M

2013-01-01

Williams syndrome (WS), a genetic, neurodevelopmental disorder, is of keen interest to music cognition researchers because of its characteristic auditory sensitivities and emotional responsiveness to music. However, actual musical perception and production abilities are more variable. We examined musicality in WS through the lens of amusia and explored how their musical perception abilities related to their auditory sensitivities, musical production skills, and emotional responsiveness to music. In our sample of 73 adolescents and adults with WS, 11% met criteria for amusia, which is higher than the 4% prevalence rate reported in the typically developing (TD) population. Amusia was not related to auditory sensitivities but was related to musical training. Performance on the amusia measure strongly predicted musical skill but not emotional responsiveness to music, which was better predicted by general auditory sensitivities. This study represents the first time amusia has been examined in a population with a known neurodevelopmental genetic disorder with a range of cognitive abilities. Results have implications for the relationships across different levels of auditory processing, musical skill development, and emotional responsiveness to music, as well as the understanding of gene-brain-behavior relationships in individuals with WS and TD individuals with and without amusia.

Bilateral vocal cord paralysis and hypothyroidism as presenting symptoms of Williams-Beuren syndrome: a case report.

Science.gov (United States)

Koren, Ilana; Kessel, Ira; Rotschild, Avi; Cohen-Kerem, Raanan

2015-09-01

Williams-Beuren syndrome is a rare neurodevelopmental disorder caused by deletion of 1.5-1.8Mb genes on chromosome 7q11.23. The syndrome was first described as a triad of supra-valvular aortic stenosis, mental retardation, and distinctive facial features. Our patient was referred due to audible inspiratory stridor when he was seven days old. Following endoscopy he was diagnosed with bilateral vocal cord paralysis and was eventually intubated due to respiratory de-compensation followed by tracheotomy. On further workup he was diagnosed with hypothyroidism. Genetic workup supported the diagnosis of Williams-Beuren syndrome. We report here a case with an unusual clinical presentation. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

33 CFR 167.1703 - In Prince William Sound: Valdez Arm Traffic Separation Scheme.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false In Prince William Sound: Valdez Arm Traffic Separation Scheme. 167.1703 Section 167.1703 Navigation and Navigable Waters COAST GUARD... Description of Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1703 In Prince...

Heterogeneity of Social Approach Behaviour in Williams Syndrome: The Role of Response Inhibition

Science.gov (United States)

Little, Katie; Riby, Deborah M.; Janes, Emily; Clark, Fiona; Fleck, Ruth; Rodgers, Jacqui

2013-01-01

The developmental disorder of Williams syndrome (WS) is associated with an overfriendly personality type, including an increased tendency to approach strangers. This atypical social approach behaviour (SAB) has been linked to two potential theories: the amygdala hypothesis and the frontal lobe hypothesis. The current study aimed to investigate…

Individualizing Services, Individualizing Responsibility

DEFF Research Database (Denmark)

Garsten, Christina; Hollertz, Katarina; Jacobsson, Kerstin

possibilities for individual voice, autonomy and self-determination in the local delivery of activation policy? What barriers do specific organisational models and practices imply for clients to choose, determine and access tailor-made programmes and services? What policy technologies are at work in governing......-oriented, and the normative demands placed on individuals appear increasingly totalizing, concerning the whole individual rather than the job-related aspects only. The paper is based on 23 in-depth interviews with individual clients as well as individual caseworkers and other professionals engaged in client-related work...

Zagreb Conference on English Contrastive Projects, 7-9 December 1970. Papers and Discussion. Studies 4. The Yugoslav Serbo-Croatian-English Contrastive Project.

Science.gov (United States)

Filipovic, Rudolf, Ed.

The fourth volume in this series contains the papers presented at the Zagreb Conference on English Contrastive Projects. They are: "Recent Center Activities in Contrastive Linguistics," by William Nemser; "The Yugoslav Serbo-Croatian-English Contrastive Project So Far," by Rudolf Filipovic; "The Poznan Polish-English Contrastive Project," by Jacek…

Pamidronate Rescue Therapy for Hypercalcemia in a Child With Williams Syndrome

Directory of Open Access Journals (Sweden)

Sami A. Sanjad

2018-05-01

Full Text Available A 15-month-old male infant diagnosed with Williams Syndrome (WS was admitted with severe hypercalcemia and nephrocalcinosis. Intravenous hydration and furosemide failed to yield an appreciable and sustainable fall in serum calcium, while the injection of pamidronate achieved a significant decrease in serum calcium in a short period of time. This bisphosphonate could be considered as a second-line treatment for refractory hypercalcemia in WS.

The development of perceptual grouping in infants with Williams syndrome

OpenAIRE

Farran, E. K.; Brown, J. H.; Cole, V. L.; Houston-Price, C.; Karmiloff-Smith, A.

2007-01-01

Perceptual grouping by luminance similarity and by proximity was investigated in infants with Williams syndrome (WS) aged between 6 and 36 months (visit 1, N=29). WS infants who were still under 36 months old, 8 months later, repeated the testing procedure (visit 2, N=15). Performance was compared to typically developing (TD) infants aged from 2 to 20 months (N=63). Consistent with the literature, TD participants showed grouping by luminance at the youngest testing age, 2 months. Grouping by ...

William Osler's "The Nervousness of American Women".

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Golden, Richard L

2008-02-01

Almost a century ago, William Osler, the foremost physician of his time, was approached by a leading periodical to write a series of articles on the health of the American woman. Osler, then the Regius Professor of Medicine at Oxford, wrote an essay dealing with the psychological stresses affecting the "new woman" of the early 20th century at varying stages of her development and the "nervousness" that ensued. The article was never published as a result of his belated reservations on the propriety of a professional writing for a lay journal. Osler's thinking frequently reflected the spirit of his Victorian-Edwardian era, although at times he demonstrated advanced and prescient ideas about sexuality, not often the subject of discussion, even in circumspect form, in contemporary nonprofessional literature.

A Mystic in English Literature: William Blake

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Ali Fahri DOĞAN

2015-12-01

Full Text Available Human beings have never been satisfied with this ephemeral world. Perhaps, yearning and desire of rejoining −stemming from the descent from the heaven to the earth− are the emotions felt by the members of both celestial and non-celestial religions. Mysticism, having started with the zeal of people who weren‘t satisfied with this ephemeral world towards the eternal world, aimed at the love of God in the religions where there is a belief of single God. In this article, glancing at the life of a Christian mystic William Blake, we will try to shed light into his mystic thoughts. While studying Blake‘s mystic thoughts, there will be common points with Sufism. Nevertheless, analysis of these common points has been assigned to other studies.

[Diagnosis of a case with Williams-Beuren syndrome with nephrocalcinosis using chromosome microarray analysis].

Science.gov (United States)

Jin, S J; Liu, M; Long, W J; Luo, X P

2016-12-02

Objective: To explore the clinical phenotypes and the genetic cause for a boy with unexplained growth retardation, nephrocalcinosis, auditory anomalies and multi-organ/system developmental disorders. Method: Routine G-banding and chromosome microarray analysis were applied to a child with unexplained growth retardation, nephrocalcinosis, auditory anomalies and multi-organ/system developmental disorders treated in the Department of Pediatrics of Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology in September 2015 and his parents to conduct the chromosomal karyotype analysis and the whole genome scanning. Deleted genes were searched in the Decipher and NCBI databases, and their relationships with the clinical phenotypes were analyzed. Result: A six-month-old boy was refered to us because of unexplained growth retardation and feeding intolerance.The affected child presented with abnormal manifestation such as special face, umbilical hernia, growth retardation, hypothyroidism, congenital heart disease, right ear sensorineural deafness, hypercalcemia and nephrocalcinosis. The child's karyotype was 46, XY, 16qh + , and his parents' karyotypes were normal. Chromosome microarray analysis revealed a 1 436 kb deletion on the 7q11.23(72701098_74136633) region of the child. This region included 23 protein-coding genes, which were reported to be corresponding to Williams-Beuren syndrome and its certain clinical phenotypes. His parents' results of chromosome microarray analysis were normal. Conclusion: A boy with characteristic manifestation of Williams-Beuren syndrome and rare nephrocalcinosis was diagnosed using chromosome microarray analysis. The deletion on the 7q11.23 might be related to the clinical phenotypes of Williams-Beuren syndrome, yet further studies are needed.

Thinking inside the box: Spatial frames of reference for drawing in Williams syndrome and typical development.

Science.gov (United States)

Hudson, Kerry D; Farran, Emily K

2017-09-01

Successfully completing a drawing relies on the ability to accurately impose and manipulate spatial frames of reference for the object that is being drawn and for the drawing space. Typically developing (TD) children use cues such as the page boundary as a frame of reference to guide the orientation of drawn lines. Individuals with Williams syndrome (WS) typically produce incohesive drawings; this is proposed to reflect a local processing bias. Across two studies, we provide the first investigation of the effect of using a frame of reference when drawing simple lines and shapes in WS and TD groups (matched for non-verbal ability). Individuals with WS (N=17 Experiment 1; N=18 Experiment 2) and TD children matched by non-verbal ability drew single lines (Experiment One) and whole shapes (Experiment Two) within a neutral, incongruent or congruent frame. The angular deviation of the drawn line/shape, relative to the model line/shape, was measured. Both groups were sensitive to spatial frames of reference when drawing single lines and whole shapes, imposed by a frame around the drawing space. A local processing bias in WS cannot explain poor drawing performance in WS. Crown Copyright © 2017. Published by Elsevier Ltd. All rights reserved.

(Amusicality in Williams syndrome: Examining relationships among auditory perception, musical skill, and emotional responsiveness to music

Directory of Open Access Journals (Sweden)

Miriam eLense

2013-08-01

Full Text Available Williams syndrome (WS, a genetic, neurodevelopmental disorder, is of keen interest to music cognition researchers because of its characteristic auditory sensitivities and emotional responsiveness to music. However, actual musical perception and production abilities are more variable. We examined musicality in WS through the lens of amusia and explored how their musical perception abilities related to their auditory sensitivities, musical production skills, and emotional responsiveness to music. In our sample of 73 adolescents and adults with WS, 11% met criteria for amusia, which is higher than the 4% prevalence rate reported in the typically developing population. Amusia was not related to auditory sensitivities but was related to musical training. Performance on the amusia measure strongly predicted musical skill but not emotional responsiveness to music, which was better predicted by general auditory sensitivities. This study represents the first time amusia has been examined in a population with a known neurodevelopmental genetic disorder with a range of cognitive abilities. Results have implications for the relationships across different levels of auditory processing, musical skill development, and emotional responsiveness to music, as well as the understanding of gene-brain-behavior relationships in individuals with WS and typically developing individuals with and without amusia.

Brookhaven Lab physicist William Willis wins the 2003 W.K.H. Panofsky prize

CERN Multimedia

2003-01-01

William Willis, a senior physicist Brookhaven National Laboratory, has won the American Physical Society's 2003 W.K.H. Panofsky Prize in Experimental Particle Physics. He received the prize, which consists of $5,000 and a certificate citing his contributions to physics, at the APS meeting in Philadelphia on April 6 (1 page).

Referential Communication Skills of Children with Williams Syndrome: Understanding when Messages Are Not Adequate

Science.gov (United States)

John, Angela E.; Rowe, Melissa L.; Mervis, Carolyn B.

2009-01-01

Although children with Williams syndrome have relatively good structural language and concrete vocabulary abilities, they have difficulty with pragmatic aspects of language. To investigate the impact of pragmatic difficulties on listener-role referential communication, we administered a picture placement task designed to measure ability to…

Hematology and clinical chemistry of sea otters vaptured in Prince William Sound, Alaska following the Exxon Valdez Oil Spill

Science.gov (United States)

Rebar, A.H.; Ballachey, Brenda E.; Bruden, D.L.; Kloecker, K.A.

1996-01-01

Hematologic and serum chemical analyses were performed on sea otter blood samples collected from 31 adult males, 63 adult females, and 42 pups captured in western Prince William Sound (oiled area), and 12 adult males, 40 adult females, and 15 pups captured in eastern Prince William Sound (unoiled area) in 1989 and 1990. Hematologic differences between eastern and western adult males were minimal. Both hematocrits and hemoglobins were higher in western than eastern otters but the biological significance of this is equivocal. Western males had higher absolute eosinophil counts, suggesting possible systemic hypersensitivity reactions. Western males had higher serum protein and serum globulin levels than eastern males, suggesting greater antigenic stimulation (more inflammatory and/or infectious conditions). There were no differences in hematologic parameters between eastern and western female otters. Some chemistry changes were present, but the degree of difference was small. Total protein and serum globulin levels were slightly higher in western females, a finding also seen in adult males. Mean levels of liver enzymes for western females were somewhat higher than for the eastern otters, suggesting the possibility of subclinical liver disease. As a group, western pup hematocrits, hemoglobins, and red cell counts were significantly lower than those of eastern pups. From a biological perspective, these reductions were minimal but supported by individual animal data. The red cell data suggest a mild anemia in western pups; however, the degree of anemia was minimal, so that biological significance was equivocal. Other hematologic and clinical chemical differences between eastern and western pups were not striking and were also of equivocal biological significance.

Sequential egocentric navigation and reliance on landmarks in Williams syndrome and typical development

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Hannah eBroadbent

2015-02-01

Full Text Available Visuospatial difficulties in Williams syndrome (WS are well documented. Recently, research has shown that spatial difficulties in WS extend to large-scale space, particularly in coding space using an allocentric frame of reference. Typically developing (TD children and adults predominantly rely on the use of a sequential egocentric strategy to navigate a large-scale route (retracing a sequence of left-right body turns. The aim of this study was to examine whether individuals with WS are able to employ a sequential egocentric strategy to guide learning and the retracing of a route. Forty-eight TD children, aged 5, 7 and 9 years and 18 participants with WS were examined on their ability to learn and retrace routes in two (6-turn virtual environment mazes (with and without landmarks. The ability to successfully retrace a route following the removal of landmarks (use of sequential egocentric coding was also examined.Although in line with TD 5 year-olds when learning a route with landmarks, individuals with WS showed significantly greater detriment when these landmarks were removed, relative to all TD groups. Moreover, the WS group made significantly more errors than all TD groups when learning a route that never contained landmarks. On a perceptual view-matching task, results revealed a high level of performance across groups, indicative of an ability to use this visual information to potentially aid navigation. These findings suggest that individuals with WS rely on landmarks to a greater extent than TD children, both for learning a route and for retracing a recently learned route. TD children, but not individuals with WS, were able to fall back on the use of a sequential egocentric strategy to navigate when landmarks were not present. Only TD children therefore coded sequential route information simultaneously with landmark information. The results are discussed in relation to known atypical cortical development and perceptual-matching abilities

Periodic Reactions: The Early Works of William C. Bray and Alfred J. Lotka

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Cervellati, Rinaldo; Greco, Emanuela

2017-01-01

Oscillating chemical reactions in the homogeneous phase have been studied intensively only since the mid-1960s, but they were known since 1920, having as forerunners the chemist William C. Bray and an "atypical" chemist Alfred J. Lotka. This contribution is the result of a careful reading of their literature and patient research into…

Performance on the Kaufman Brief Intelligence Test-2 by Children with Williams Syndrome

Science.gov (United States)

Pitts, C. Holley; Mervis, Carolyn B.

2016-01-01

We describe the performance of 292 4- to 17-year-olds with Williams syndrome (WS) on the Kaufman Brief Intelligence Test-2 (KBIT-2; Kaufman & Kaufman, 2004). Mean IQ Composite, Verbal standard score (SS), and Nonverbal SS were in the borderline range relative to the general population, with variability similar to the general population.…

Atypical Verbal Communication Pattern According to Others' Attention in Children with Williams Syndrome

Science.gov (United States)

Asada, Kosuke; Tomiwa, Kiyotaka; Okada, Masako; Itakura, Shoji

2010-01-01

Children with Williams syndrome (WS) have been reported to often face problems in daily communication and to have deficits in their pragmatic language abilities. To test this hypothesis, we examined whether children with WS could modify their verbal communication according to others' attention in order to share what they did. The children with WS…

Williams syndrome-specific neuroanatomical profile and its associations with behavioral features.

Science.gov (United States)

Fan, Chun Chieh; Brown, Timothy T; Bartsch, Hauke; Kuperman, Joshua M; Hagler, Donald J; Schork, Andrew; Searcy, Yvonne; Bellugi, Ursula; Halgren, Eric; Dale, Anders M

2017-01-01

Williams Syndrome (WS) is a rare genetic disorder with unique behavioral features. Yet the rareness of WS has limited the number and type of studies that can be conducted in which inferences are made about how neuroanatomical abnormalities mediate behaviors. In this study, we extracted a WS-specific neuroanatomical profile from structural magnetic resonance imaging (MRI) measurements and tested its association with behavioral features of WS. Using a WS adult cohort (22 WS, 16 healthy controls), we modeled a sparse representation of a WS-specific neuroanatomical profile. The predictive performances are robust within the training cohort (10-fold cross-validation, AUC = 1.0) and accurately identify all WS individuals in an independent child WS cohort (seven WS, 59 children with diverse developmental status, AUC = 1.0). The WS-specific neuroanatomical profile includes measurements in the orbitofrontal cortex, superior parietal cortex, Sylvian fissures, and basal ganglia, and variability within these areas related to the underlying size of hemizygous deletion in patients with partial deletions. The profile intensity mediated the overall cognitive impairment as well as personality features related to hypersociability. Our results imply that the unique behaviors in WS were mediated through the constellation of abnormalities in cortical-subcortical circuitry consistent in child WS and adult WS. The robustness of the derived WS-specific neuroanatomical profile also demonstrates the potential utility of our approach in both clinical and research applications.

Testing inter-observer reliability of the Transition Analysis aging method on the William M. Bass forensic skeletal collection.

Science.gov (United States)

Fojas, Christina L; Kim, Jieun; Minsky-Rowland, Jocelyn D; Algee-Hewitt, Bridget F B

2018-01-01

Skeletal age estimation is an integral part of the biological profile. Recent work shows how multiple-trait approaches better capture senescence as it occurs at different rates among individuals. Furthermore, a Bayesian statistical framework of analysis provides more useful age estimates. The component-scoring method of Transition Analysis (TA) may resolve many of the functional and statistical limitations of traditional phase-aging methods and is applicable to both paleodemography and forensic casework. The present study contributes to TA-research by validating TA for multiple, differently experienced observers using a collection of modern forensic skeletal cases. Five researchers independently applied TA to a random sample of 58 documented individuals from the William M. Bass Forensic Skeletal Collection, for whom knowledge of chronological age was withheld. Resulting scores were input into the ADBOU software and maximum likelihood estimates (MLEs) and 95% confidence intervals (CIs) were produced using the forensic prior. Krippendorff's alpha was used to evaluate interrater reliability and agreement. Inaccuracy and bias were measured to gauge the magnitude and direction of difference between estimated ages and chronological ages among the five observers. The majority of traits had moderate to excellent agreement among observers (≥0.6). The superior surface morphology had the least congruence (0.4), while the ventral symphyseal margin had the most (0.9) among scores. Inaccuracy was the lowest for individuals younger than 30 and the greatest for individuals over 60. Consistent over-estimation of individuals younger than 30 and under-estimation of individuals over 40 years old occurred. Individuals in their 30s showed a mixed pattern of under- and over-estimation among observers. These results support the use of the TA method by researchers of varying experience levels. Further, they validate its use on forensic cases, given the low error overall. © 2017 Wiley

Genome rearrangements detected by SNP microarrays in individuals with intellectual disability referred with possible Williams syndrome.

Directory of Open Access Journals (Sweden)

Ariel M Pani

2010-08-01

Full Text Available Intellectual disability (ID affects 2-3% of the population and may occur with or without multiple congenital anomalies (MCA or other medical conditions. Established genetic syndromes and visible chromosome abnormalities account for a substantial percentage of ID diagnoses, although for approximately 50% the molecular etiology is unknown. Individuals with features suggestive of various syndromes but lacking their associated genetic anomalies pose a formidable clinical challenge. With the advent of microarray techniques, submicroscopic genome alterations not associated with known syndromes are emerging as a significant cause of ID and MCA.High-density SNP microarrays were used to determine genome wide copy number in 42 individuals: 7 with confirmed alterations in the WS region but atypical clinical phenotypes, 31 with ID and/or MCA, and 4 controls. One individual from the first group had the most telomeric gene in the WS critical region deleted along with 2 Mb of flanking sequence. A second person had the classic WS deletion and a rearrangement on chromosome 5p within the Cri du Chat syndrome (OMIM:123450 region. Six individuals from the ID/MCA group had large rearrangements (3 deletions, 3 duplications, one of whom had a large inversion associated with a deletion that was not detected by the SNP arrays.Combining SNP microarray analyses and qPCR allowed us to clone and sequence 21 deletion breakpoints in individuals with atypical deletions in the WS region and/or ID or MCA. Comparison of these breakpoints to databases of genomic variation revealed that 52% occurred in regions harboring structural variants in the general population. For two probands the genomic alterations were flanked by segmental duplications, which frequently mediate recurrent genome rearrangements; these may represent new genomic disorders. While SNP arrays and related technologies can identify potentially pathogenic deletions and duplications, obtaining sequence information

William Crabtree and the date of Easter

Science.gov (United States)

Sellers, D.

2014-04-01

A previously unknown treatise by William Crabtree (c.1603-c.1644) has recently been unearthed in the Lancashire Record Office. The treatise, in manuscript form and written in 1640, deals with the controversy over the long-term impact of the Julian Calendar - then in use in England - upon the ecclesiastical dating of Easter. By Crabtree's time, the timing of the Easter celebration in England was often several weeks adrift of the intentions of the early Church Fathers. The Gregorian Calendar, which Roman Catholic countries had adopted as long ago as 1582 in order to resolve the problem, was still vehemently resisted by the English state. This is possibly the only surviving manuscript in Crabtree's own hand. In it, he displays noteworthy dispassionate objectivity as he outlines the astronomical basis for the Easter date and explains why it has gone awry.

Errors of Measurement, Theory, and Public Policy. William H. Angoff Memorial Lecture Series

Science.gov (United States)

Kane, Michael

2010-01-01

The 12th annual William H. Angoff Memorial Lecture was presented by Dr. Michael T. Kane, ETS's (Educational Testing Service) Samuel J. Messick Chair in Test Validity and the former Director of Research at the National Conference of Bar Examiners. Dr. Kane argues that it is important for policymakers to recognize the impact of errors of measurement…

William Allen Miller (1817-70): a distinguished scientist re-discovered.

Science.gov (United States)

Ashley-Miller, Michael

2008-11-01

Dr William Allen Miller developed an early interest in science and astronomy at secondary school. Although qualifying in medicine, he pursued a career in Chemistry at King's College, London. A particular interest in spectrum analysis led to a collaboration with Dr Huggins in examining the spectra of stars. For this work they each received the Gold Medal of the Royal Astronomical Society. His subsequent work at King's College, the Royal Society, the Courts of Law and for various Government enterprises earned him an outstanding scientific and advisory reputation.

Pulmonary Arterial Stent Implantation in an Adult with Williams Syndrome

International Nuclear Information System (INIS)

Reesink, Herre J.; Henneman, Onno D. F.; Delden, Otto M. van; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

2007-01-01

We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent implantation. Short-term follow-up showed a good clinical result with excellent patency of the stents but early restenosis of the segments in which only balloon angioplasty was performed. These stenoses were subsequently also treated successfully by stent implantation. Stent patency was observed 3 years after the first procedure

Dr. William O. Coffee and his absorption cure for cataract.

Science.gov (United States)

Ferry, A P

1989-08-01

Dr. William O. Coffee was an ophthalmologist who conducted an office and mail-order practice in the Midwest from the 1880s until 1927. His main stock in trade was a self-discovered absorption cure for a variety of ocular diseases, with particular emphasis on the medical cure of cataracts. Dr. Coffee's career was a checkered one, marked by dubious credentials, exuberant self-promotion, unlikely and exaggerated claims of medical successes, plagiarism, and rejection by the medical "establishment." Certain parallels may be drawn between his activities and some currently observed practices in ophthalmology.

[Sir William Richard Gowers: author of the "bible of neurology"].

Science.gov (United States)

Hirose, Genjiro

2014-11-01

William Richard Gowers is one of the great pioneers in neurology and the author of the well-known neurology textbook, "A Manual of Diseases of the Nervous System." His concepts of neurology are based on meticulously and carefully accumulated knowledge of history, observations, and neurological examinations of patients with various neurological diseases. He is not only a great neurologist but also a great teacher who loves teaching students and physicians through well-prepared lectures. We can glean the essence of the field of neurology through his life story and numerous writings concerning neurological diseases.

A gestalt approach to the science fiction novels of William Gibson

OpenAIRE

McFarlane, Anna M.

2015-01-01

Gestalt psychologists Kurt Koffka and Wolfgang Köhler argue that human perception relies on a form, or gestalt, into which perceptions are assimilated. Gestalt theory has been applied to the visual arts by Rudolf Arnheim and to literature by Wolfgang Iser. My original contribution to knowledge is to use gestalt theory to perform literary criticism, an approach that highlights the importance of perception in William Gibson’s novels and the impact of this emphasis on posthumanism and science fi...

Brief Report: Exploring the Relationship between Sensory Processing and Repetitive Behaviours in Williams Syndrome

Science.gov (United States)

Riby, Deborah M.; Janes, Emily; Rodgers, Jacqui

2013-01-01

This study explored the relationship between sensory processing abnormalities and repetitive behaviours in children with Williams Syndrome (WS; n = 21). This is a novel investigation bringing together two clinical phenomena for the first time in this neuro-developmental disorder. Parents completed the Sensory Profile (Short Form; Dunn in The…

William Blake’s Milton a Poem as a conversion narrative in the Behmenist tradition

DEFF Research Database (Denmark)

Jessen, Elisabeth Engell

2014-01-01

The term ‘conversion narrative’ lacks proper definition and can be understood more broadly than is often the case, underlining its fictive nature. I show this by reading William Blake’s Milton a Poem as a conversion narrative, exploring how Blake weaves a wider discourse of conversion around...

Psychopathology and behavior problems in children and adolescents with Williams syndrome: Distinctive relationships with cognition.

Science.gov (United States)

Osório, Ana A C; Rossi, Natália F; Gonçalves, Óscar F; Sampaio, Adriana; Giacheti, Célia M

2017-08-01

Several studies have documented the high prevalence of psychopathology and behavior problems in Williams syndrome (WS). However, the links between cognitive development and such symptoms need further clarification. Our study aims to expand current knowledge on levels of behavior problems and its links to cognition in a sample of Brazilian individuals with WS. A total of 25 children and adolescents with WS and their parents participated in this study. The participants' IQs were assessed with the Wechsler Scales of Intelligence (for children or adults) and parental reports of psychopathology/behavior problems were collected using the Child Behavior Checklist (CBCL). The presence of clinically significant attention problems was a main feature in our sample of children and adolescents with WS. In the children, higher IQ scores were found to be significantly associated with less externalizing problems, while in the adolescents cognitive abilities were found to be associated with less internalizing symptoms. These results provide further insight into the links between psychopathology and behavior problems and cognitive abilities in WS, and suggest the need to take age into consideration when analyzing such relationships.

Surgical repair of supravalvular aortic stenosis in children with williams syndrome: a 30-year experience.

Science.gov (United States)

Fricke, Tyson A; d'Udekem, Yves; Brizard, Christian P; Wheaton, Gavin; Weintraub, Robert G; Konstantinov, Igor E

2015-04-01

Williams syndrome is an uncommon genetic disorder associated with supravalvular aortic stenosis (SVAS) in childhood. We reviewed outcomes of children with Williams syndrome who underwent repair of SVAS during a 30-year period at a single institution. Between 1982 and 2012, 28 patients with Williams syndrome were operated on for SVAS. Mean age at operation was 5.2 years (range, 3 months to 13 years), and mean weight at operation was 18.6 kg (range, 4.1 to 72.4 kg). Associated cardiac lesions in 11 patients (39.3%) were repaired at the time of the SVAS repair. The most common associated cardiac lesion was main pulmonary artery stenosis (8 of 28 [28%]). A 3-patch repair was performed in 10 patients, a Doty repair in 17, and a McGoon repair in 1 (3.6%). There were no early deaths. Follow-up was 96% complete (27 of 28). Overall mean follow-up was 11.2 years (range, 1 month to 27.3 years). Mean follow-up was 5 years (range, 1 month to 14.3 years) for the 3-patch repair patients and 14.7 years (range, 6 weeks to 27 years) for the Doty repair patients. Of the 17 Doty patients, there were 4 (24%) late deaths, occurring at 6 weeks, 3.5 years, 4 years, and 16 years after the initial operation. There were no late deaths in the 3-patch repair patients. Overall survival was 86% at 5, 10, and 15 years after repair. Survival was 82% at 5, 10 and 15 years for the Doty repair patients. Overall, 6 of 27 patients (22%) patients required late reoperation at a mean of 11.2 years (range, 3.6 to 23 years). No 3-patch repair patients required reoperation. Overall freedom from reoperation was 91% at 5 years and 73% at 10 and 15 years. Freedom from reoperation for the Doty repair patients was 93% at 5 years and 71% at 10 and 15 years. Surgical repair of SVAS in children Williams syndrome has excellent early results. However, significant late mortality and morbidity warrants close follow-up. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights

Syntax in Spanish-speaking children with Williams syndrome.

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Benítez-Burraco, Antonio; Garayzábal, Elena; Cuetos, Fernando

2016-01-01

The syntactic skills of Spanish-speaking children with Williams syndrome (WS) were assessed in different areas (phrase structure, recursion, and bound anaphora). Children were compared to typically-developing peers matched either in chronological age (CA-TD) or in verbal age (VA-TD). In all tasks children with WS performed significantly worse than CA-TD children, but similarly to VA-TD children. However, significant differences were observed in specific domains, particularly regarding sentences with cross-serial dependencies. At the same time, children with WS were less sensitive to syntactic constraints and exhibited a poorer knowledge of some functional words (specifically, of nonreflexive pronouns). A processing bottleneck or a computational constraint may account for this outcome. Copyright © 2016 Elsevier Inc. All rights reserved.

The other Dr Hooker: William Dawson Hooker (1816-40).

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Lambert, Harold

2011-11-01

William Hooker and his son Joseph were famous as botanists and as the creators of the Royal Botanic Gardens in Kew. Joseph was famous also as the friend and mentor of Charles Darwin. But there was another brother, a little older than Joseph, also a doctor and naturalist. He went to Jamaica in the interests of his health and soon died there of yellow fever. His life was short and tragic with a medical conundrum at its end but its story also illustrates many of the beliefs and concerns that preoccupied doctors in this early Victorian era. It also illustrates the close relationship between medicine and botany that prevailed then.

Developmental Trajectories of Structural and Pragmatic Language Skills in School-Aged Children with Williams Syndrome

Science.gov (United States)

Van Den Heuvel, E.; Manders, E.; Swillen, A.; Zink, I.

2016-01-01

Background: This study aimed to compare developmental courses of structural and pragmatic language skills in school-aged children with Williams syndrome (WS) and children with idiopathic intellectual disability (IID). Comparison of these language trajectories could highlight syndrome-specific developmental features. Method: Twelve monolingual…

Sensitivity of the autonomic nervous system to visual and auditory affect across social and non-social domains in Williams syndrome

Directory of Open Access Journals (Sweden)

Anna Maaria Järvinen

2012-09-01

Full Text Available Although individuals with Williams syndrome (WS typically demonstrate an increased appetitive social drive, their social profile is characterized by dissociations, including socially fearless behavior coupled with anxiousness, and distinct patterns of peaks and valleys of ability. The aim of this study was to compare the processing of social and non-social visually and aurally presented affective stimuli, at the levels of behavior and autonomic nervous system (ANS responsivity, in individuals with WS contrasted with a typically developing (TD group, with the view of elucidating the highly sociable and emotionally sensitive predisposition noted in WS. Behavioral findings supported previous studies of enhanced competence in processing social over non-social stimuli by individuals with WS; however, the patterns of ANS functioning underlying the behavioral performance revealed a surprising profile previously undocumented in WS. Specifically, increased heart rate (HR reactivity, and a failure for electrodermal activity (EDA to habituate were found in individuals with WS contrasted with the TD group, predominantly in response to visual social-affective stimuli. Within the auditory domain, greater arousal linked to variation in heart beat period was observed in relation to music stimuli in individuals with WS. Taken together, the findings suggest that the pattern of ANS response in WS is more complex than previously noted, with increased arousal to face and music stimuli potentially underpinning the heightened behavioral emotionality to such stimuli. The lack of habituation may underlie the increased affiliation and attraction to faces characterizing individuals with WS. Future research directions are suggested.

Sensitivity of the Autonomic Nervous System to Visual and Auditory Affect Across Social and Non-Social Domains in Williams Syndrome

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Järvinen, Anna; Dering, Benjamin; Neumann, Dirk; Ng, Rowena; Crivelli, Davide; Grichanik, Mark; Korenberg, Julie R.; Bellugi, Ursula

2012-01-01

Although individuals with Williams syndrome (WS) typically demonstrate an increased appetitive social drive, their social profile is characterized by dissociations, including socially fearless behavior coupled with anxiousness, and distinct patterns of “peaks and valleys” of ability. The aim of this study was to compare the processing of social and non-social visually and aurally presented affective stimuli, at the levels of behavior and autonomic nervous system (ANS) responsivity, in individuals with WS contrasted with a typically developing (TD) group, with the view of elucidating the highly sociable and emotionally sensitive predisposition noted in WS. Behavioral findings supported previous studies of enhanced competence in processing social over non-social stimuli by individuals with WS; however, the patterns of ANS functioning underlying the behavioral performance revealed a surprising profile previously undocumented in WS. Specifically, increased heart rate (HR) reactivity, and a failure for electrodermal activity to habituate were found in individuals with WS contrasted with the TD group, predominantly in response to visual social affective stimuli. Within the auditory domain, greater arousal linked to variation in heart beat period was observed in relation to music stimuli in individuals with WS. Taken together, the findings suggest that the pattern of ANS response in WS is more complex than previously noted, with increased arousal to face and music stimuli potentially underpinning the heightened behavioral emotionality to such stimuli. The lack of habituation may underlie the increased affiliation and attraction to faces characterizing individuals with WS. Future research directions are suggested. PMID:23049519

Interpreting "Mind-Cure": William James and the "chief task…of the science of human nature".

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Sutton, Emma Kate

2012-01-01

The private papers of the philosopher-psychologist, William James, indicate that he frequented several mental healers during his life, undertaking 100-200 therapeutic sessions concerning a range of symptoms from angina to insomnia. The success of the mind-cure movement constituted for James both a corroboration, and an extension, of the new research into the subconscious self and the psychogenesis of disease. Epistemologically, the experiences of those converts to the "mind-cure religion" exemplified his conviction that positivistic scientific enquiry can only reveal only one part of a wider reality. Metaphysically their reports comprised a powerful body of support for the existence of a "higher consciousness," a supernatural world of some description. The positing of such a source of "supernormal" healing power was, for James, the best way to reconcile the accounts of those who had been regenerated, via their faith, despite having exhausted all natural reserves of energy and will. © 2012 Wiley Periodicals, Inc.

(A)musicality in Williams syndrome: examining relationships among auditory perception, musical skill, and emotional responsiveness to music

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Lense, Miriam D.; Shivers, Carolyn M.; Dykens, Elisabeth M.

2013-01-01

Williams syndrome (WS), a genetic, neurodevelopmental disorder, is of keen interest to music cognition researchers because of its characteristic auditory sensitivities and emotional responsiveness to music. However, actual musical perception and production abilities are more variable. We examined musicality in WS through the lens of amusia and explored how their musical perception abilities related to their auditory sensitivities, musical production skills, and emotional responsiveness to music. In our sample of 73 adolescents and adults with WS, 11% met criteria for amusia, which is higher than the 4% prevalence rate reported in the typically developing (TD) population. Amusia was not related to auditory sensitivities but was related to musical training. Performance on the amusia measure strongly predicted musical skill but not emotional responsiveness to music, which was better predicted by general auditory sensitivities. This study represents the first time amusia has been examined in a population with a known neurodevelopmental genetic disorder with a range of cognitive abilities. Results have implications for the relationships across different levels of auditory processing, musical skill development, and emotional responsiveness to music, as well as the understanding of gene-brain-behavior relationships in individuals with WS and TD individuals with and without amusia. PMID:23966965

Recognition of Emotional and Nonemotional Facial Expressions: A Comparison between Williams Syndrome and Autism

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Lacroix, Agnes; Guidetti, Michele; Roge, Bernadette; Reilly, Judy

2009-01-01

The aim of our study was to compare two neurodevelopmental disorders (Williams syndrome and autism) in terms of the ability to recognize emotional and nonemotional facial expressions. The comparison of these two disorders is particularly relevant to the investigation of face processing and should contribute to a better understanding of social…

Prosodic Abilities in Spanish and English Children with Williams Syndrome: A Cross-Linguistic Study

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Martinez-Castilla, Pastora; Stojanovik, Vesna; Setter, Jane; Sotillo, Maria

2012-01-01

The aim of this study was to compare the prosodic profiles of English- and Spanish-speaking children with Williams syndrome (WS), examining cross-linguistic differences. Two groups of children with WS, English and Spanish, of similar chronological and nonverbal mental age, were compared on performance in expressive and receptive prosodic tasks…

Developments in fiber-positioning technology for the WEAVE instrument at the William Herschel Telescope

NARCIS (Netherlands)

Schallig, Ellen; Lewis, Ian J.; Gilbert, James; Dalton, Gavin; Brock, Matthew; Abrams, Don Carlos; Middleton, Kevin; Aguerri, J. Alfonso L.; Bonifacio, Piercarlo; Carrasco, Esperanza; Trager, Scott C.; Vallenari, Antonella

2016-01-01

WEAVE is the next-generation wide-field optical spectroscopy facility for the William Herschel Telescope (WHT) on La Palma in the Canary Islands, Spain. It is a multi-object "pick-and-place" fibre-fed spectrograph with a 1000 fibre multiplex behind a new dedicated 2° prime focus corrector. The WEAVE

Readings on the Comedies (William Shakespeare). The Greenhaven Press Literary Companion to British Literature.

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Swisher, Clarice, Ed.

Intended as an accessible resource for students researching William Shakespeare's (1564-1616) "Comedies," this collection of essays about the great playwright contains an in-depth biography and writings taken from a wide variety of sources. The essays are edited to accommodate the reading and comprehension levels of young adults; each…

Effectiveness of commercial microbial products in enhancing oil degradation in Prince William Sound field plots

International Nuclear Information System (INIS)

Venosa, A.D.; Haines, J.R.; Allen, D.M.

1991-01-01

In the spring of 1990, previously reported laboratory experiments were conducted on 10 commercial microbial products to test for enhanced biodegradation of weathered crude oil from the Exxon Valdez oil spill. The laboratory tests measured the rate and extent of oil degradation in closed flasks. Weathered oil from the beaches in Alaska and seawater from Prince William Sound were used in the tests. Two of the 10 products were found to provide significantly greater alkane degradation than flasks supplemented with mineral nutrients alone. These two products were selected for further testing on a beach in Prince William Sound. A randomized complete block experiment was designed to compare the effectiveness of these two products in enhancing oil degradation compared to simple fertilizer alone. Four small plots consisting of a no nutrient control, a mineral nutrient plot, and two plots receiving mineral nutrients plus the two products, were laid out on a contaminated beach. These four plots comprised a 'block' of treatments, and this block was replicated four times on the same beach. Triplicate samples of beach sediment were collected at four equally spaced time intervals and analyzed for oil residue weight and alkane hydrocarbon profile changes with time. The objective was to determine if either of the two commercial microbiological products was able to enhance bioremediation of an oil-contaminated beach in Prince William Sound to an extent greater than that achievable by simple fertilizer application. Results indicated no significant differences among the four treatments in the 27-day period of the experiment

Trajectories and models of individual growth

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Arseniy Karkach

2006-11-01

Full Text Available It has long been recognized that the patterns of growth play an important role in the evolution of age trajectories of fertility and mortality (Williams, 1957. Life history studies would benefit from a better understanding of strategies and mechanisms of growth, but still no comparative research on individual growth strategies has been conducted. Growth patterns and methods have been shaped by evolution and a great variety of them are observed. Two distinct patterns - determinate and indeterminate growth - are of a special interest for these studies since they present qualitatively different outcomes of evolution. We attempt to draw together studies covering growth in plant and animal species across a wide range of phyla focusing primarily on the noted qualitative features. We also review mathematical descriptions of growth, namely empirical growth curves and growth models, and discuss the directions of future research.

The Dance of the Dead Rhino: William Kentridge’s Magic Flute

OpenAIRE

Guarracino, Serena

2010-01-01

The article offers a reading of the staging of The Magic Flute by visual artist William Kentridge, focusing on his introduction of the rhino in the visual landscape of the opera as symbol for the silenced subject of violence. Operatic tradition has always been concerned with the staging of death, in particular with the death of its female protagonists, and recent scholarship has highlighted the complicity of the genre with the ideology of Western patriarchy and colonial violence. In this ligh...

Ian D. Copestake, The Ethics of William Carlos Williams’s Poetry.

Directory of Open Access Journals (Sweden)

Aristotle University of Greece

2011-09-01

Full Text Available Ian D. Copestake’s monograph on William Carlos Williams’s poetry offers a well-informed and well-documented insight into the connection between Williams’s writing with Unitarianism and Emersonian thinking. In this very well-written and accessible book, the reader gets introduced to a number of poems in addition to excerpts from Williams’s essays, letters and autobiography which facilitate the understanding and appreciation of the poet’s attempt to promote “independent thought and action” (5....

Welcome Donald Trump, Baltics! The presidency to endure? / Mark Hertling, William E. Pomeranz ; intervjueeris Linas Jegelevicius

Index Scriptorium Estoniae

Hertling, Mark

2017-01-01

USA Euroopa vägede endine ülem kindralleitnant Mark Hertlingi ja Kennani Instituudi asedirektor William E. Pomeranz analüüsivad Ameerika Ühendriikide uue presidendi Tonald Trumpi ametisseasumise võimalikku mõju Balti riikide julgeolekule