11 results
Even simple framing effects are rational
- Stephen J. Flusberg, Paul H. Thibodeau, Kevin J. Holmes
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- Journal:
- Behavioral and Brain Sciences / Volume 45 / 2022
- Published online by Cambridge University Press:
- 25 October 2022, e228
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Bermúdez persuasively argues that framing effects are not as irrational as commonly supposed. In focusing on the reasoning of individual decision-makers in complex situations, however, he neglects the crucial role of the social-communicative context for eliciting certain framing effects. We contend that many framing effects are best explained in terms of basic, rational principles of discourse processing and pragmatic reasoning.
16 - Predicting Individual Differences in Cognitive Ability from Brain Imaging and Genetics
- from Part IV - Predictive Modeling Approaches
- Edited by Aron K. Barbey, University of Illinois, Urbana-Champaign, Sherif Karama, McGill University, Montréal, Richard J. Haier, University of California, Irvine
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- The Cambridge Handbook of Intelligence and Cognitive Neuroscience
- Published online:
- 11 June 2021
- Print publication:
- 01 July 2021, pp 327-348
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Summary
The study of intelligence, or general cognitive ability, is one of the earliest avenues of modern psychological enquiry (Spearman, 1904). A consistent goal of this field is the development of cognitive measures that predict real-world outcomes, ranging from academic performance, health (Calvin et al., 2017), and psychopathology (Woodberry, Giuliano, & Seidman, 2008), to mortality and morbidity rates (Batty, Deary, & Gottfredson, 2007). Despite evidence linking intelligence with a host of important life outcomes, we remain far from a mechanistic understanding of how neurobiological processes contribute to individual differences in general cognitive ability. Excitingly for researchers, advances in predictive statistical modeling, the emergence of well-powered imaging and genetic datasets, and a cultural shift toward open access data may allow for behavioral prediction at the level of a single individual (Miller et al., 2016; Poldrack & Gorgolewski, 2014).
Slowed Temporal and Parietal Cerebrovascular Response in Patients with Alzheimer’s Disease
- Kenneth R. Holmes, David Tang-Wai, Kevin Sam, Larissa McKetton, Julien Poublanc, Adrian P. Crawley, Olivia Sobczyk, Melanie Cohn, James Duffin, Maria Carmela Tartaglia, Sandra E. Black, Joseph A. Fisher, Bruce Wasserman, David J. Mikulis
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- Journal:
- Canadian Journal of Neurological Sciences / Volume 47 / Issue 3 / May 2020
- Published online by Cambridge University Press:
- 13 February 2020, pp. 366-373
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Background:
Recent investigations now suggest that cerebrovascular reactivity (CVR) is impaired in Alzheimer’s disease (AD) and may underpin part of the disease’s neurovascular component. However, our understanding of the relationship between the magnitude of CVR, the speed of cerebrovascular response, and the progression of AD is still limited. This is especially true in patients with mild cognitive impairment (MCI), which is recognized as an intermediate stage between normal aging and dementia. The purpose of this study was to investigate AD and MCI patients by mapping repeatable and accurate measures of cerebrovascular function, namely the magnitude and speed of cerebrovascular response (τ) to a vasoactive stimulus in key predilection sites for vascular dysfunction in AD.
Methods:Thirty-three subjects (age range: 52–83 years, 20 males) were prospectively recruited. CVR and τ were assessed using blood oxygen level-dependent MRI during a standardized carbon dioxide stimulus. Temporal and parietal cortical regions of interest (ROIs) were generated from anatomical images using the FreeSurfer image analysis suite.
Results:Of 33 subjects recruited, 3 individuals were excluded, leaving 30 subjects for analysis, consisting of 6 individuals with early AD, 11 individuals with MCI, and 13 older healthy controls (HCs). τ was found to be significantly higher in the AD group compared to the HC group in both the temporal (p = 0.03) and parietal cortex (p = 0.01) following a one-way ANCOVA correcting for age and microangiopathy scoring and a Bonferroni post-hoc correction.
Conclusion:The study findings suggest that AD is associated with a slowing of the cerebrovascular response in the temporal and parietal cortices.
The North Atlantic Fish Revolution (ca. AD 1500)
- Poul Holm, Francis Ludlow, Cordula Scherer, Charles Travis, Bernard Allaire, Cristina Brito, Patrick W. Hayes, J. Al Matthews, Kieran J. Rankin, Richard J. Breen, Robert Legg, Kevin Lougheed, John Nicholls
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- Journal:
- Quaternary Research / Volume 108 / July 2022
- Published online by Cambridge University Press:
- 17 April 2019, pp. 92-106
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We propose the concept of the “Fish Revolution” to demarcate the dramatic increase in North Atlantic fisheries after AD 1500, which led to a 15-fold increase of cod (Gadus morhua) catch volumes and likely a tripling of fish protein to the European market. We consider three key questions: (1) What were the environmental parameters of the Fish Revolution? (2) What were the globalising effects of the Fish Revolution? (3) What were the consequences of the Fish Revolution for fishing communities? While these questions would have been considered unknowable a decade or two ago, methodological developments in marine environmental history and historical ecology have moved information about both supply and demand into the realm of the discernible. Although much research remains to be done, we conclude that this was a major event in the history of resource extraction from the sea, mediated by forces of climate change and globalisation, and is likely to provide a fruitful agenda for future multidisciplinary research.
Outcrop analysis and facies model of an Upper Permian tidally influenced fluvio-deltaic system: Northern Sydney Basin, SE Australia
- Jake Breckenridge, Angelos G. Maravelis, Octavian Catuneanu, Kevin Ruming, Erin Holmes, William J. Collins
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- Journal:
- Geological Magazine / Volume 156 / Issue 10 / October 2019
- Published online by Cambridge University Press:
- 07 March 2019, pp. 1715-1741
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An integrated study of sedimentological, sequence-stratigraphic and palaeodispersal analysis was applied to the Upper-Permian clastic sedimentary succession in the Northern Sydney Basin, Australia. The succession is subdivided into fifteen facies and three facies associations. The facies associations are further subdivided into eight sub-facies associations. The sedimentary evolution involves progradation from delta-front to delta-plain to fluvial depositional environments, with a significant increase in sediment grain size across the unconformable contact that separates the deltaic from the overlying fluvial system. In contrast to the delta front that is wave/storm- and/or river-influenced, the delta plain is significantly affected by tides, with the impact of tidal currents decreasing up-sequence in the delta plain. The general lack of wave-influenced sedimentary structures suggests low wave energy in the delta plain. The abrupt termination of the tidal impact in the fluvial realm relates to the steep topographic gradients and high sediment supply, which accompanied the uplift of the New England Orogen. The sequence-stratigraphic framework includes highstand (deltaic forest and topset) and lowstand (fluvial topset) systems tracts, separated by a subaerial unconformity. In contrast to most of the mud-rich modern counterparts, this is an example of a sand-rich tidally influenced deltaic system, developed adjacent to the source region. This investigation presents a depositional model for tidal successions in regions of tectonic uplift and confinement.
Right idea, wrong magnitude system
- Stella F. Lourenco, Lauren S. Aulet, Vladislav Ayzenberg, Chi-Ngai Cheung, Kevin J. Holmes
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- Journal:
- Behavioral and Brain Sciences / Volume 40 / 2017
- Published online by Cambridge University Press:
- 27 July 2017, e177
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Leibovich et al. claim that number representations are non-existent early in life and that the associations between number and continuous magnitudes reside in stimulus confounds. We challenge both claims – positing, instead, that number is represented independently of continuous magnitudes already in infancy, but is nonetheless more deeply connected to other magnitudes through adulthood than acknowledged by the “sense of magnitude” theory.
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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Spatial language as a window on representations of three-dimensional space
- Kevin J. Holmes, Phillip Wolff
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- Behavioral and Brain Sciences / Volume 36 / Issue 5 / October 2013
- Published online by Cambridge University Press:
- 08 October 2013, pp. 550-551
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Recent research investigating the language–thought interface in the spatial domain points to representations of the horizontal and vertical dimensions that closely resemble those posited by Jeffery et al. However, the findings suggest that such representations, rather than being tied to navigation, may instead reflect more general properties of the perception of space.
Notes on Contributors
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- By Chris Beyers, Amy Branam, Alan Brown, Mark Canada, Patricia A. Cunningham, Satwik Dasgupta, David Dowling, John Evelev, Michael J. Everton, Benjamin F. Fisher, Paul Fisher, Meghan A. Freeman, Christopher Gair, Andrea Goulet, Jonathan Hartmann, Kevin J. Hayes, Gregory Hays, Alvin Holm, Lindsey Hursh, James M. Hutchisson, Paul Christian Jones, Katherine Kim, Nathaniel Lewis, Bruce Mills, Travis Montgomery, Tara Moore, Bran Nicol, Philip Edward Phillips, Anne Boyd Rioux, Therese M. Rizzo, Kathryn K. Shinn, Heidi Silcox, Peter Swirski, Jonathan Taylor, John Tresch, Lois Davis Vines, Jeffrey Andrew Weinstock, Brett Zimmerman
- Edited by Kevin J. Hayes, University of Central Oklahoma
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- Book:
- Edgar Allan Poe in Context
- Published online:
- 05 November 2013
- Print publication:
- 29 October 2012, pp ix-xiv
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Host-range testing of a mixture of two nucleopolyhedroviruses of Choristoneura fumiferana (Lepidoptera: Tortricidae)
- William J. Kaupp, Kevin N. Barber, William E. Fick, Peter M. Ebling, Tim R. Ladd, Stephen B. Holmes
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- Journal:
- The Canadian Entomologist / Volume 143 / Issue 2 / April 2011
- Published online by Cambridge University Press:
- 03 January 2012, pp. 165-177
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The host range of a mixture of Choristoneura fumiferana (Clemens) nucleopolyhedroviruses (CfMNPV and CfDefNPV) was investigated using a per os bioassay of larvae of 29 species of Lepidoptera and adult males of Megachile rotundata (F.) (Hymenoptera: Megachilidae). Using a whole-genomic DNA probe, positive results were obtained in 8 of 10 Tortricidae: Archips cerasivorana (Fitch), Choristoneura fractivittana (Clemens), C. fumiferana, Choristoneura occidentalis Freeman, Choristoneura pinus pinus Freeman, Choristoneura rosaceana (Harris), Clepsis persicana (Fitch), and Cydia pomonella (L.); one Crambidae: Ostrinia nubilalis (Hübner); one arctiine Erebidae: Estigmene acrea (Drury); and two Noctuidae: Oligia illocata (Walker) and Pyrrhia exprimens (Walker). Mortality rates were highest among C. fumiferana, C. occidentalis, C. pinus pinus, A. cerasivorana, and C. pomonella. Sequenced polymerase chain reaction (PCR) amplicons from infected individuals from several species confirmed that the primer sets amplified the target viruses. CfMNPV was consistently found in virus-fed C. fumiferana; whereas, CfDefNPV was present only occasionally. The presence of CfMNPV and CfDefNPV in A. cerasivorana was confirmed by PCR and DNA sequencing. Significant treatment-mortality rates were induced in the noctuids P. exprimens and Acronicta impleta Walker; PCR determined that both viruses were present in treated P. exprimens but only CfMNPV was present in A. impleta. No virus was detected in M. rotundata.
Contemporary management of congenital malformations of the heart in infants with Ellis – van Creveld syndrome: a report of nine cases
- Matthew J. O’Connor, Nicholas L. Rider, R. Thomas Collins II, Brian D. Hanna, D. Holmes Morton, Kevin A. Strauss
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- Journal:
- Cardiology in the Young / Volume 21 / Issue 2 / April 2011
- Published online by Cambridge University Press:
- 12 November 2010, pp. 145-152
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Introduction
Ellis – van Creveld syndrome is an autosomal recessive disorder manifest by short-limb dwarfism, thoracic dystrophy, postaxial polydactyly, dysplastic nails and teeth, and an approximately 60% incidence of congenital malformations of the heart. Despite patients with Ellis – van Creveld syndrome being regarded as having a high surgical risk, few data are available regarding their outcomes following surgery for congenital malformations of the heart in the current era.
Materials and methodsIn this retrospective report, we summarise the clinical observations and outcomes of nine infants with Ellis – van Creveld syndrome who underwent surgery for congenital malformations of the heart between 2004 and 2009.
ResultsWe identified 15 patients with Ellis – van Creveld syndrome during the study period; 11 (73%) had haemodynamically significant congenital malformations of the heart warranting surgery. In two of these patients, surgery was not performed. Of the nine patients who underwent surgery, all of whom were infants, eight (89%) had various forms of an atrioventricular septal defect and one patient (11%) had hypoplastic left heart syndrome (mitral and aortic atresia). Among the nine patients who underwent surgery, four (44%) died at a median of 102 days with a range of 25–149 days post-operatively, mostly from respiratory failure. Respiratory morbidity was seen in all surviving patients, of whom three underwent tracheostomy.
ConclusionsSurgery for congenital malformations of the heart can be successful in infants with Ellis – van Creveld syndrome, but mortality is high and post-operative respiratory morbidity should be expected.