2,574 research outputs found
Neuroacanthocytosis Syndromes
Full text linkNeuroacanthocytosis (NA) syndromes are a group of genetically defined diseases characterized by the association of red blood cell acanthocytosis and progressive degeneration of the basal ganglia. NA syndromes are exceptionally rare with an estimated prevalence of less than 1 to 5 per 1'000'000 inhabitants for each disorder. The core NA syndromes include autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome which have a Huntington's disease-like phenotype consisting of a choreatic movement disorder, psychiatric manifestations and cognitive decline, and additional multi-system features including myopathy and axonal neuropathy. In addition, cardiomyopathy may occur in McLeod syndrome. Acanthocytes are also found in a proportion of patients with autosomal dominant Huntington's disease-like 2, autosomal recessive pantothenate kinase-associated neurodegeneration and several inherited disorders of lipoprotein metabolism, namely abetalipoproteinemia (Bassen-Kornzweig syndrome) and hypobetalipoproteinemia leading to vitamin E malabsorption. The latter disorders are characterized by a peripheral neuropathy and sensory ataxia due to dorsal column degeneration, but movement disorders and cognitive impairment are not present. NA syndromes are caused by disease-specific genetic mutations. The mechanism by which these mutations cause neurodegeneration is not known. The association of the acanthocytic membrane abnormality with selective degeneration of the basal ganglia, however, suggests a common pathogenetic pathway. Laboratory tests include blood smears to detect acanthocytosis and determination of serum creatine kinase. Cerebral magnetic resonance imaging may demonstrate striatal atrophy. Kell and Kx blood group antigens are reduced or absent in McLeod syndrome. Western blot for chorein demonstrates absence of this protein in red blood cells of chorea-acanthocytosis patients. Specific genetic testing is possible in all NA syndromes. Differential diagnoses include Huntington disease and other causes of progressive hyperkinetic movement disorders. There are no curative therapies for NA syndromes. Regular cardiologic studies and avoidance of transfusion complications are mandatory in McLeod syndrome. The hyperkinetic movement disorder may be treated as in Huntington disease. Other symptoms including psychiatric manifestations should be managed in a symptom-oriented manner. NA syndromes have a relentlessly progressive course usually over two to three decades
Indonesia : democracy and the promise of good governance/ edit.: Ross H. McLeod; Andrew MacIntyre
No full textThe costs of reducing deforestation in Indonesia
No full textIn this second contribution to the Policy Dialogue, Colin Hunt emphasises the large contribution that oil palm plantations and the pulp and paper industry have been making to Indonesia's economic growth in recent years, notwithstanding the environmental consequences of such activities. The implication is that avoided deforestation can be expected to have a significant negative impact on segments of the population who would benefit from the business and employment opportunities that would otherwise be generated, directly or indirectly. Palm oil companies typically spend about three dollars on goods, services and labour for every dollar of profit. The author argues that any compensation package for avoided deforestation needs to include all the potential beneficiaries of palm oil production, not just the palm oil companies, and to generate economic activity similar to that being replaced. (Ed.)
A Conversation with Ross Brann
No full textRoss Brann studied at the University of California-Berkeley, the Hebrew University-Jerusalem, New York University, and the American University in Cairo. He has taught at Cornell since 1986 and served nineteen years as Chair of the Department of Near Eastern Studies. Professor Brann is the author of The Compunctious Poet: Cultural Ambiguity and Hebrew Poetry in Muslim Spain (Johns Hopkins University Press, 1991) and Power in the Portrayal: Representations of Muslims and Jews in Islamic Spain (Princeton University Press, 2002). He has received fellowships from the John Simon Guggenheim Memorial Foundation, the National Endowment for the Humanities, the Center for Advanced Judaic Studies of the University of Pennsylvania, and the Frankel Center for Advanced Judaic Studies at the University of Michigan. Brann is also the editor of four volumes and author of essays on the intersection of medieval Jewish and Islamic cultures. In 2019 he completed Andalusi Moorings: Al-Andalus and Sefarad as Tropes of Islamic and Jewish Culture and submitted it for publication to the University of Pennsylvania Press. In 2007 Brann was appointed Stephen H. Weiss Presidential Fellow and in June 2010 he stepped down as the faculty co-chair of the West Campus House System Council after six years of service as the founding Alice Cook House Professor-Dean.1_hgsoa28
Trail A.A.A. Junior Smoke Eaters, B.C. champions, Western Canada champions, Memorial Cup finalists, 1943-44
No full textClockwise from top left: I. Toffolo, B.Bentley, G.M. Thomson (coach), W.O. Williams (President), F. Turik, H. Magliani, R. Kelly, R. Butler, H. McLeod, I. McLeod, L. DePaolis, J. Duffield, H. Ross, C. Marquess, H. WIlson, E. Miller, J. Miller. Centre photos: L. Demore (coach), J. Decembrini (trainer), J.H. Shields (Secretary), R. Koehle (mascot)
McLeod myopathy revisited: more neurogenic and less benign
No full textThe X-linked McLeod neuroacanthocytosis syndrome (MLS) has originally been denoted as ‘benign' McLeod myopathy. We assessed the clinical findings and the muscle pathology in the eponymous index patient, Hugh McLeod, and in nine additional MLS patients. Only one patient had manifested with neuromuscular symptoms. During a mean follow-up of 15 years, however, eight patients including the initial index patient showed elevated skeletal muscle creatine kinase levels ranging from 300 to 3000 U/L, and had developed muscle weakness and atrophy. Two patients had disabling leg weakness. Muscle histology was abnormal in all 10 patients. Clear but unspecific myopathic changes were found in only four patients. All patients, however, had neurogenic changes of variable degree. Post-mortem motor and sensory nerve examinations support the view that muscle atrophy and weakness are predominantly due to an axonal motor neuropathy rather than to a primary myopathy. Multisystem manifestations developed in eight patients at a mean age of 39 years. Three patients manifested with psychiatric features comprising schizophrenia-like psychosis and personality disorder, two presented with generalized seizures and one with chorea. During follow-up, seven patients developed chorea, six had psychiatric disorders, five had cognitive decline and three had generalized seizures. Five patients died because of MLS-related complications including sudden cardiac death, chronic heart failure and pneumonia between 55 and 69 years. In conclusion, our findings confirm that MLS is not a benign condition but rather a progressive multisystem disorder sharing many features with Huntington's diseas
Asia’s Economic Transformation: Implications for Australia
Full text linkThis policy brief details recent economic developments
in four of Asia’s largest economies - Japan, India, China
and Indonesia - and assesses the implications of these
changes for Australia. Jenny Corbett looks at how economic
frustrations led to political change in Japan and argues that,
despite this year’s devastating earthquake and tsunami,
there is opportunity for continued economic growth if the
Japanese government, in conjunction with industry, pursues
a program of smart rebuilding and does not become mired
in prolonged political disagreement. Raghbendra Jha looks
at how structural changes in the Indian economy, along with
changes in Indian society, have increased the rate of growth
in the world’s second largest country. He argues that while
trade and investment relations between Australia and India
are strong, there is substantial room for improvement. He
highlights infrastructure and the services sector as potential
expansion areas. Ligang Song sees continued benefits to
Australia from China’s ongoing growth in the third part
of this policy brief. This rapid growth, however, will bring
macroeconomic, environmental, and social challenges in
China that can only be overcome through major structural
reforms. Finally, Ross McLeod examines the issues confronting
Indonesia’s economy, including high inflation, corruption
and wasteful energy use. He argues that while these present
significant challenges, Indonesia’s economy is still performing
strongly, being one of the few countries not to have suffered
a severe decline in growth as a result of the Global Financial
Crisis
David Ross Locke: Forgotten Editor
No full text Petroleum Vesuvius Nasby is a well-known literary figure, but his creator, David Ross Locke, has received little attention for his editorship of the Toledo Blade and other journalistic accomplishments. The author brings both the flavor of the Nasby letters and a resumé of Locke's success in Toledo. </jats:p
Aqueous aesthetics : an art history of change
No full textThis essay, the edited text of a lecture by Ross Gibson, demonstrates what its author calls an "aqueous aesthetics", and in the process calls for an art history that "records, analyses and theorises how creativity proceeds in a world that is suffused with fluidity". The structure and language of Gibson's text embodies this fluidity, as do the various objects—art works, music, literature, chance encounters, anecdotes—he incorporates as his examples. Adopting a voice at once personal and learned, Gibson proposes a mode of history writing different in kind from that usually encountered in books about art, a writing that is multidisciplinary in its range of interests and transnational in its scope
Dr. Hugh H. Smythe (1913-1977): anthropologist and statesman, 1979
Full text linkProblems Dr. Hugh Heyne Smith (August 19, 1913 - June 22, 1977) attained distinction as an anthropologist with broad interests in all of the social sciences and as a stateman who represented the United States as Ambassador to two countries, Syria and Malta. This thesis is a pioneer attempt to assemble data for a review and assessment of his career as scientist and statesman. Purpose In addition to the above, the study of the life and career of Dr. Smythe provides material for the understanding of the professional growth and development of black scholars who elect to pursue careers in sociology and anthropology. Procedure Dr. Hugh H. Smythe was a prolific writer, contributing numerous articles in the fields of anthropology, sociology, intergroup relations, and political science to many scholarly, and also some popular, periodicals. He was co-author with his wife, Mabel Murphy Smith, of the book, The New Nigerian Elite, and contributed to several others. The first task is to discover and to read his contributions to the literature. Results A preliminary collation and summary of many of the works authored by Dr. Smythe has been achieved. This should be of great assistance to future scholars who shall wish to pursue further studies of the life and career or Dr Hugh H. Smythe, when his papers which have been deposited with the Library of Congress are made available to the public
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