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B.02 Recessive mutations in ATP8A2 cause severe hypotonia, cognitive impairment, hyperkinetic movement disorders and progressive optic atrophy
- HJ McMillan, A Telegrafi, A Singleton, M Cho, D Lelli, FC Lynn, J Griffin, A Asamoah, T Rinne, CE Erasmus, DA Koolen, CA Haaxma, B Keren, D Doummar, C Mignot, I Thompson, L Velsher, M Dehghani, M Vahidi Mehrjardi, R Maroofian, M Tchan, C Simons, J Christodoulou, E Martín-Hernández, MJ Guillen Sacoto, LB Henderson, H McLaughlin, LL Molday, RS Molday, G Yoon
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- Journal:
- Canadian Journal of Neurological Sciences / Volume 45 / Issue s2 / June 2018
- Published online by Cambridge University Press:
- 27 June 2018, p. S12
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Background:ATP8A2 mutations have only recently been associated with human disease. We present the clinical features from the largest cohort of patients with this disorder reported to date. Methods: An observational study of 9 unreported and 2 previously reported patients with biallelic ATP8A2 mutations was carried out at multiple centres. Results: The mean age of the cohort was 9.4 years old (range: 2.5-28 yrs). All patients demonstrated developmental delay, severe hypotonia and movement disorders: chorea/choreoathetosis (100%), dystonia (27%) or facial dyskinesia (18%). Hypotonia was apparent at birth (70%) or before 6 months old (100%). Optic atrophy was observed in 75% of patients who had a funduscopic examination. MRI of the brain was normal for most patients with a small proportion showing mild cortical atrophy (30%), delayed myelination (20%) and/or hypoplastic optic nerves (20%). Epilepsy was seen in two older patients. Conclusions:ATP8A2 gene mutations have emerged as a cause of a novel phenotype characterized by developmental delay, severe hypotonia and hyperkinetic movement disorders. Optic atrophy is common and may only become apparent in the first few years of life, necessitating repeat ophthalmologic evaluation. Early recognition of the cardinal features of this condition will facilitate diagnosis of this disorder.
At the Movies: Contemporary Australian Indigenous Cultural Expressions – Transforming the Australian Story
- Lynn Griffin, Steven Griffin, Michelle Trudgett
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- Journal:
- The Australian Journal of Indigenous Education / Volume 47 / Issue 2 / December 2018
- Published online by Cambridge University Press:
- 21 June 2017, pp. 131-138
- Print publication:
- December 2018
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Cinema is an art form widely recognised as an agent to change the social condition and alter traditional norms. Movies can be used to educate and transform society's collective conscience. Indigenous Australian artists utilise the power of artistic expression as a tool to initiate change in the attitudes and perceptions of the broader Australian society. Australia's story has predominately been told from the coloniser's viewpoint. This narrative is being rewritten through Indigenous artists utilising the power of cinema to create compelling stories with Indigenous control. This medium has come into prominence for Indigenous Australians to express our culture, ontology and politics. Movies such as Samson and Delilah, Bran Nue Dae, The Sapphires and Rabbit-Proof Fence for example, have highlighted the injustices of past policies, adding new dimensions to the Australian narrative. These three films are just a few of the Indigenous Australian produced films being used in the Australian National Curriculum.
Through this medium, Australian Indigenous voices are rewriting the Australian narrative from the Indigenous perspective, deconstructing the predominant stereotypical perceptions of Indigenous culture and reframing the Australian story. Films are essential educational tools to cross the cultural space that often separates Indigenous learners from their non-Indigenous counterparts.
Comparing Techniques for Quantifying Haloxyfop Absorption and Translocation in Johnsongrass (Sorghum halepense)
- Robert S. Peregoy, Lynn M. Kitchen, James L. Griffin, Michael P. Braverman
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- Journal:
- Weed Technology / Volume 9 / Issue 3 / September 1995
- Published online by Cambridge University Press:
- 12 June 2017, pp. 462-467
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In comparing laboratory techniques, absorption and translocation of 14C-haloxyfop was not affected by droplet size or treatment area. Treatment with haloxyfop prior to application of 14C-haloxyfop resulted in 27% less translocation of radiolabel from the treated leaf and 43% less acropetal translocation compared with plants receiving only the radiolabeled herbicide. Leaf washing with distilled water, distilled water plus haloxyfop, distilled water plus surfactant, distilled water plus haloxyfop plus surfactant, 100% ethanol, 100% methanol, or methanol:water (1:1 v/v) removed similar amounts of radiolabel, while chloroform removed more. Considering some of the differences found with these techniques, pretreatment with nonlabeled herbicide should be routine practice and water used as the standard solvent.
Moisture Stress Effects on the Absorption, Translocation, and Metabolism of Haloxyfop in Johnsongrass (Sorghum halepense) and Large Crabgrass (Digitaria sanguinalis)
- Robert S. Peregoy, Lynn M. Kitchen, Peter W. Jordan, James L. Griffin
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- Journal:
- Weed Science / Volume 38 / Issue 4-5 / September 1990
- Published online by Cambridge University Press:
- 12 June 2017, pp. 331-337
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Glasshouse studies were undertaken to determine the effect of imposed moisture stress on the phytotoxicity of haloxyfop; the absorption, translocation, and metabolism of 14C-haloxyfop; and 14C-photoassimilate partitioning in johnsongrass and large crabgrass. Following foliar applications of haloxyfop at 30 and 25 g ai ha–1 to large crabgrass and johnsongrass, respectively, control 15 days after treatment was 92% for nonstressed plants and less than 12% for water-stressed plants. Foliar absorption of 14C-haloxyfop was reduced by moisture stress 1, 3, 5, and 24 h after treatment (HAT) in large crabgrass and 1, 3, 5, 48, and 72 HAT in johnsongrass. Regardless of stress treatment, absorption in both species reached a maximum by 24 HAT. Translocation of the radiolabel from the treated leaf to plant parts above and below the node of the treated leaf was inhibited by moisture stress in large crabgrass and johnsongrass at all harvest intervals beginning 5 and 24 HAT, respectively. Metabolism of 14C-haloxyfop was not altered by moisture stress. Fixation of 14CO2 and subsequent distribution of the 14C-photoassimilates were reduced by moisture stress. Decreases in photoassimilate translocation were similar to reductions in 14C-haloxyfop translocation. Moisture stress reduced the phytotoxicity of haloxyfop in the two grasses, and the reduced activity of haloxyfop appeared to be partially related to changes in herbicide absorption and translocation.
Contributors
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- By Mitchell Aboulafia, Frederick Adams, Marilyn McCord Adams, Robert M. Adams, Laird Addis, James W. Allard, David Allison, William P. Alston, Karl Ameriks, C. Anthony Anderson, David Leech Anderson, Lanier Anderson, Roger Ariew, David Armstrong, Denis G. Arnold, E. J. Ashworth, Margaret Atherton, Robin Attfield, Bruce Aune, Edward Wilson Averill, Jody Azzouni, Kent Bach, Andrew Bailey, Lynne Rudder Baker, Thomas R. Baldwin, Jon Barwise, George Bealer, William Bechtel, Lawrence C. Becker, Mark A. Bedau, Ernst Behler, José A. Benardete, Ermanno Bencivenga, Jan Berg, Michael Bergmann, Robert L. Bernasconi, Sven Bernecker, Bernard Berofsky, Rod Bertolet, Charles J. Beyer, Christian Beyer, Joseph Bien, Joseph Bien, Peg Birmingham, Ivan Boh, James Bohman, Daniel Bonevac, Laurence BonJour, William J. Bouwsma, Raymond D. Bradley, Myles Brand, Richard B. Brandt, Michael E. Bratman, Stephen E. Braude, Daniel Breazeale, Angela Breitenbach, Jason Bridges, David O. Brink, Gordon G. Brittan, Justin Broackes, Dan W. Brock, Aaron Bronfman, Jeffrey E. Brower, Bartosz Brozek, Anthony Brueckner, Jeffrey Bub, Lara Buchak, Otavio Bueno, Ann E. Bumpus, Robert W. Burch, John Burgess, Arthur W. Burks, Panayot Butchvarov, Robert E. Butts, Marina Bykova, Patrick Byrne, David Carr, Noël Carroll, Edward S. Casey, Victor Caston, Victor Caston, Albert Casullo, Robert L. Causey, Alan K. L. Chan, Ruth Chang, Deen K. Chatterjee, Andrew Chignell, Roderick M. Chisholm, Kelly J. Clark, E. J. Coffman, Robin Collins, Brian P. Copenhaver, John Corcoran, John Cottingham, Roger Crisp, Frederick J. Crosson, Antonio S. Cua, Phillip D. Cummins, Martin Curd, Adam Cureton, Andrew Cutrofello, Stephen Darwall, Paul Sheldon Davies, Wayne A. Davis, Timothy Joseph Day, Claudio de Almeida, Mario De Caro, Mario De Caro, John Deigh, C. F. Delaney, Daniel C. Dennett, Michael R. DePaul, Michael Detlefsen, Daniel Trent Devereux, Philip E. Devine, John M. Dillon, Martin C. Dillon, Robert DiSalle, Mary Domski, Alan Donagan, Paul Draper, Fred Dretske, Mircea Dumitru, Wilhelm Dupré, Gerald Dworkin, John Earman, Ellery Eells, Catherine Z. Elgin, Berent Enç, Ronald P. Endicott, Edward Erwin, John Etchemendy, C. Stephen Evans, Susan L. Feagin, Solomon Feferman, Richard Feldman, Arthur Fine, Maurice A. Finocchiaro, William FitzPatrick, Richard E. Flathman, Gvozden Flego, Richard Foley, Graeme Forbes, Rainer Forst, Malcolm R. Forster, Daniel Fouke, Patrick Francken, Samuel Freeman, Elizabeth Fricker, Miranda Fricker, Michael Friedman, Michael Fuerstein, Richard A. Fumerton, Alan Gabbey, Pieranna Garavaso, Daniel Garber, Jorge L. A. Garcia, Robert K. Garcia, Don Garrett, Philip Gasper, Gerald Gaus, Berys Gaut, Bernard Gert, Roger F. Gibson, Cody Gilmore, Carl Ginet, Alan H. Goldman, Alvin I. Goldman, Alfonso Gömez-Lobo, Lenn E. Goodman, Robert M. Gordon, Stefan Gosepath, Jorge J. E. Gracia, Daniel W. Graham, George A. Graham, Peter J. Graham, Richard E. Grandy, I. Grattan-Guinness, John Greco, Philip T. Grier, Nicholas Griffin, Nicholas Griffin, David A. Griffiths, Paul J. Griffiths, Stephen R. Grimm, Charles L. Griswold, Charles B. Guignon, Pete A. Y. Gunter, Dimitri Gutas, Gary Gutting, Paul Guyer, Kwame Gyekye, Oscar A. Haac, Raul Hakli, Raul Hakli, Michael Hallett, Edward C. Halper, Jean Hampton, R. James Hankinson, K. R. Hanley, Russell Hardin, Robert M. Harnish, William Harper, David Harrah, Kevin Hart, Ali Hasan, William Hasker, John Haugeland, Roger Hausheer, William Heald, Peter Heath, Richard Heck, John F. Heil, Vincent F. Hendricks, Stephen Hetherington, Francis Heylighen, Kathleen Marie Higgins, Risto Hilpinen, Harold T. Hodes, Joshua Hoffman, Alan Holland, Robert L. Holmes, Richard Holton, Brad W. Hooker, Terence E. Horgan, Tamara Horowitz, Paul Horwich, Vittorio Hösle, Paul Hoβfeld, Daniel Howard-Snyder, Frances Howard-Snyder, Anne Hudson, Deal W. Hudson, Carl A. Huffman, David L. Hull, Patricia Huntington, Thomas Hurka, Paul Hurley, Rosalind Hursthouse, Guillermo Hurtado, Ronald E. Hustwit, Sarah Hutton, Jonathan Jenkins Ichikawa, Harry A. Ide, David Ingram, Philip J. Ivanhoe, Alfred L. Ivry, Frank Jackson, Dale Jacquette, Joseph Jedwab, Richard Jeffrey, David Alan Johnson, Edward Johnson, Mark D. Jordan, Richard Joyce, Hwa Yol Jung, Robert Hillary Kane, Tomis Kapitan, Jacquelyn Ann K. Kegley, James A. Keller, Ralph Kennedy, Sergei Khoruzhii, Jaegwon Kim, Yersu Kim, Nathan L. King, Patricia Kitcher, Peter D. Klein, E. D. Klemke, Virginia Klenk, George L. Kline, Christian Klotz, Simo Knuuttila, Joseph J. Kockelmans, Konstantin Kolenda, Sebastian Tomasz Kołodziejczyk, Isaac Kramnick, Richard Kraut, Fred Kroon, Manfred Kuehn, Steven T. Kuhn, Henry E. Kyburg, John Lachs, Jennifer Lackey, Stephen E. Lahey, Andrea Lavazza, Thomas H. Leahey, Joo Heung Lee, Keith Lehrer, Dorothy Leland, Noah M. Lemos, Ernest LePore, Sarah-Jane Leslie, Isaac Levi, Andrew Levine, Alan E. Lewis, Daniel E. Little, Shu-hsien Liu, Shu-hsien Liu, Alan K. L. Chan, Brian Loar, Lawrence B. Lombard, John Longeway, Dominic McIver Lopes, Michael J. Loux, E. J. Lowe, Steven Luper, Eugene C. Luschei, William G. Lycan, David Lyons, David Macarthur, Danielle Macbeth, Scott MacDonald, Jacob L. Mackey, Louis H. Mackey, Penelope Mackie, Edward H. Madden, Penelope Maddy, G. B. Madison, Bernd Magnus, Pekka Mäkelä, Rudolf A. Makkreel, David Manley, William E. Mann (W.E.M.), Vladimir Marchenkov, Peter Markie, Jean-Pierre Marquis, Ausonio Marras, Mike W. Martin, A. P. Martinich, William L. McBride, David McCabe, Storrs McCall, Hugh J. McCann, Robert N. McCauley, John J. McDermott, Sarah McGrath, Ralph McInerny, Daniel J. McKaughan, Thomas McKay, Michael McKinsey, Brian P. McLaughlin, Ernan McMullin, Anthonie Meijers, Jack W. Meiland, William Jason Melanson, Alfred R. Mele, Joseph R. Mendola, Christopher Menzel, Michael J. Meyer, Christian B. Miller, David W. Miller, Peter Millican, Robert N. Minor, Phillip Mitsis, James A. Montmarquet, Michael S. Moore, Tim Moore, Benjamin Morison, Donald R. Morrison, Stephen J. Morse, Paul K. Moser, Alexander P. D. Mourelatos, Ian Mueller, James Bernard Murphy, Mark C. Murphy, Steven Nadler, Jan Narveson, Alan Nelson, Jerome Neu, Samuel Newlands, Kai Nielsen, Ilkka Niiniluoto, Carlos G. Noreña, Calvin G. Normore, David Fate Norton, Nikolaj Nottelmann, Donald Nute, David S. Oderberg, Steve Odin, Michael O’Rourke, Willard G. Oxtoby, Heinz Paetzold, George S. Pappas, Anthony J. Parel, Lydia Patton, R. P. Peerenboom, Francis Jeffry Pelletier, Adriaan T. Peperzak, Derk Pereboom, Jaroslav Peregrin, Glen Pettigrove, Philip Pettit, Edmund L. Pincoffs, Andrew Pinsent, Robert B. Pippin, Alvin Plantinga, Louis P. Pojman, Richard H. Popkin, John F. Post, Carl J. Posy, William J. Prior, Richard Purtill, Michael Quante, Philip L. Quinn, Philip L. Quinn, Elizabeth S. Radcliffe, Diana Raffman, Gerard Raulet, Stephen L. Read, Andrews Reath, Andrew Reisner, Nicholas Rescher, Henry S. Richardson, Robert C. Richardson, Thomas Ricketts, Wayne D. Riggs, Mark Roberts, Robert C. Roberts, Luke Robinson, Alexander Rosenberg, Gary Rosenkranz, Bernice Glatzer Rosenthal, Adina L. Roskies, William L. Rowe, T. M. Rudavsky, Michael Ruse, Bruce Russell, Lilly-Marlene Russow, Dan Ryder, R. M. Sainsbury, Joseph Salerno, Nathan Salmon, Wesley C. Salmon, Constantine Sandis, David H. Sanford, Marco Santambrogio, David Sapire, Ruth A. Saunders, Geoffrey Sayre-McCord, Charles Sayward, James P. Scanlan, Richard Schacht, Tamar Schapiro, Frederick F. Schmitt, Jerome B. Schneewind, Calvin O. Schrag, Alan D. Schrift, George F. Schumm, Jean-Loup Seban, David N. Sedley, Kenneth Seeskin, Krister Segerberg, Charlene Haddock Seigfried, Dennis M. Senchuk, James F. Sennett, William Lad Sessions, Stewart Shapiro, Tommie Shelby, Donald W. Sherburne, Christopher Shields, Roger A. Shiner, Sydney Shoemaker, Robert K. Shope, Kwong-loi Shun, Wilfried Sieg, A. John Simmons, Robert L. Simon, Marcus G. Singer, Georgette Sinkler, Walter Sinnott-Armstrong, Matti T. Sintonen, Lawrence Sklar, Brian Skyrms, Robert C. Sleigh, Michael Anthony Slote, Hans Sluga, Barry Smith, Michael Smith, Robin Smith, Robert Sokolowski, Robert C. Solomon, Marta Soniewicka, Philip Soper, Ernest Sosa, Nicholas Southwood, Paul Vincent Spade, T. L. S. Sprigge, Eric O. Springsted, George J. Stack, Rebecca Stangl, Jason Stanley, Florian Steinberger, Sören Stenlund, Christopher Stephens, James P. Sterba, Josef Stern, Matthias Steup, M. A. Stewart, Leopold Stubenberg, Edith Dudley Sulla, Frederick Suppe, Jere Paul Surber, David George Sussman, Sigrún Svavarsdóttir, Zeno G. Swijtink, Richard Swinburne, Charles C. Taliaferro, Robert B. Talisse, John Tasioulas, Paul Teller, Larry S. Temkin, Mark Textor, H. S. Thayer, Peter Thielke, Alan Thomas, Amie L. Thomasson, Katherine Thomson-Jones, Joshua C. Thurow, Vzalerie Tiberius, Terrence N. Tice, Paul Tidman, Mark C. Timmons, William Tolhurst, James E. Tomberlin, Rosemarie Tong, Lawrence Torcello, Kelly Trogdon, J. D. Trout, Robert E. Tully, Raimo Tuomela, John Turri, Martin M. Tweedale, Thomas Uebel, Jennifer Uleman, James Van Cleve, Harry van der Linden, Peter van Inwagen, Bryan W. Van Norden, René van Woudenberg, Donald Phillip Verene, Samantha Vice, Thomas Vinci, Donald Wayne Viney, Barbara Von Eckardt, Peter B. M. Vranas, Steven J. Wagner, William J. Wainwright, Paul E. Walker, Robert E. Wall, Craig Walton, Douglas Walton, Eric Watkins, Richard A. Watson, Michael V. Wedin, Rudolph H. Weingartner, Paul Weirich, Paul J. Weithman, Carl Wellman, Howard Wettstein, Samuel C. Wheeler, Stephen A. White, Jennifer Whiting, Edward R. Wierenga, Michael Williams, Fred Wilson, W. Kent Wilson, Kenneth P. Winkler, John F. Wippel, Jan Woleński, Allan B. Wolter, Nicholas P. Wolterstorff, Rega Wood, W. Jay Wood, Paul Woodruff, Alison Wylie, Gideon Yaffe, Takashi Yagisawa, Yutaka Yamamoto, Keith E. Yandell, Xiaomei Yang, Dean Zimmerman, Günter Zoller, Catherine Zuckert, Michael Zuckert, Jack A. Zupko (J.A.Z.)
- Edited by Robert Audi, University of Notre Dame, Indiana
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- The Cambridge Dictionary of Philosophy
- Published online:
- 05 August 2015
- Print publication:
- 27 April 2015, pp ix-xxx
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Evaluation of an Interactive Electronic Health Education Tool in Rural Afghanistan
- Glen Kim, Suzanne Griffin, Hedeyat Nadem, Jawad Aria, Lynn Lawry
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- Journal:
- Prehospital and Disaster Medicine / Volume 23 / Issue 3 / June 2008
- Published online by Cambridge University Press:
- 17 February 2017, pp. 218-226
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- June 2008
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Introduction: Low education levels may limit community-based health worker (CHW) efforts in rural Afghanistan. In 2004, LeapFrog Enterprises and the United States Department of Health and Human Services developed the Afghan Family Health Book (AFHB), an interactive, electronic picture book, to communicate public health messages in rural Afghanistan. Changes in health knowledge among households exposed to the AFHB vs. CHWs were compared.
Methods: From January–June 2005, baseline and follow-up panel surveys were administered in Pashto-speaking Laghman and Dari-speaking Kabul provinces. Within each province, an AFHB and a CHW district were randomly sampled using a stratified, 2-staged cluster sample design (total 98 clusters and 3,372 households). Surveys tested knowledge of 17 health domains at baseline and on follow-up at three months. For each domain, multivariate logistic regression was used to assess the effect of the AFHB on follow-up pass rates, controlling for demographics and differences in baseline knowledge.
Results: Both AFHB and CHW resulted in statistically significant changes in pass rates on follow-up, although there were greater gains among AFHB users for five domains among Pashto-speakers (micronutrients, malaria, sexually transmitted diseases, postpartum care, and breast-feeding) and seven domains among Dari-speakers (diet, malaria, mental health, birth-spacing, and prenatal/neonatal/postpartum care). Community-based health workers effected greater knowledge gains only for the Dari breast-feeding module. Participants favored CHW over the AFHB, which they found poorly translated and difficult to use.
Conclusions: The AFHB has potential to improve public health knowledge among rural Afghans. Future efforts may benefit from involvement of local health agencies and the integration of interactive technology with traditional CHW approaches.
Unusually persistent complainants
- Grant Lester, Franzcp Beth Wilson, Lynn Griffin, Paul E. Mullen
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- Journal:
- The British Journal of Psychiatry / Volume 184 / Issue 4 / April 2004
- Published online by Cambridge University Press:
- 02 January 2018, pp. 352-356
- Print publication:
- April 2004
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Background
Querulous paranoia may have disappeared from the psychiatric literature, but is it flourishing in modern complaints organisations and the courts? Aims To investigate the unusually persistent complainants who lay waste to their own lives and place inordinate demands and stress on complaints organisations.
MethodComplaints officers completed questionnaires on both unusually persistent complainants and matched controls.
ResultsPersistent complainants (distinguished by their pursuit of vindication and retribution) consumed time and resources and resorted to both direct and veiled threats. Attempts to distinguish these people from a control group on the basis of the manner in which their claims were initially managed failed.
ConclusionsPersistent complainants' pursuit of vindication and retribution fits badly with complaints systems established to deliver reparation and compensation. These complainants damaged the financial and social fabric of their own lives and frightened those dealing with their claims. The study suggests methods of early detection and alternative management strategies.