Mucopolysaccharidosis type I (MPS I) is a rare lysosomal storage disorder treated with bone marrow transplantation or enzyme replacement therapy with laronidase, a high-cost orphan drug. Laronidase was approved by the US Food and Drug Administration and the European Medicines Agency in 2003 and by the Brazilian National Health Surveillance Agency in 2005. Many Brazilian MPS I patients have been receiving laronidase despite the absence of a governmental policy regulating access to the drug. Epidemiological and treatment data concerning MPS I (...) are scarce. This study aims to present a demographic profile of Brazilian patients with MPS I, describe the routes of access to laronidase in Brazil, and discuss associated ethical issues relating to public funding of orphan drugs. (shrink)

Listening to someone from some distance in a crowded room you may experience the following phenomenon: when looking at them speak, you may both hear and see where the source of the sounds is; but when your eyes are turned elsewhere, you may no longer be able to detect exactly where the voice must be coming from. With your eyes again fixed on the speaker, and the movement of her lips a clear sense of the source of the sound will (...) return. This ‘ventriloquist’ effect reflects the ways in which visual cognition can dominate auditory perception. And this phenomenological observation is one what you can verify or disconfirm in your own case just by the slightest reflection on what it is like for you to listen to someone with or without visual contact with them. (shrink)

MEDIEVAL LOGICS LAMBERT MARIE DE RIJK (ed.), Die mittelalterlichen Traktate De mod0 opponendiet respondendi, Einleitung und Ausgabe der einschlagigen Texte. (Beitrage zur Geschichte der Philosophie und Theologie des Mittelalters, Neue Folge Band 17.) Miinster: Aschendorff, 1980. 379 pp. No price stated. THE SEVENTEENTH CENTURY MARTA FATTORI, Lessico del Novum Organum di Francesco Bacone. Rome: Edizioni dell'Ateneo 1980. Two volumes, il + 543, 520 pp. Lire 65.000. VIVIAN SALMON, The study of language in 17th century England. (Amsterdam Studies in the Theory (...) and History of Linguistic Science, Series 111: Studies in theHistory of Linguistics, Volume 17.) Amsterdam: John Benjamins B.V., 1979.x + 218 pp. Dfl. 65. Theoria cum Praxi. Zum Verhaltnis von Theorie und Praxis im 17. und 18. Jahrhundert. (Akten des 111. Internationalen Leibnizkongress, Hannover, 12. bis 17.November 1977, Band 111: Logik, Erkenntnistheorie, Wissenschaftstheorie, Metaphysik, Theologie.) Wiesbaden: Franz Steiner Verlag, 1980. vii + 269 pp. DM 48. CLASSICAL AND NON-CLASSICAL LOGICS MICHAEL CLARK, The place of syllogistic in logical theory. Nottingham: University of Nottingham Press, 1980. ix + 151 pp. £3.00. A.F. PARKER-RHODES, The theory of indistinguishables. Dordrecht, Boston and London: D. Reidel Publishing Company, 1981. xvii + 216 pp. Dfl.90.00/$39.50. NICHOLAS RESCHER and ROBERT BRANDOM, The logic of inconsistency. Oxford:Basil Blackwell, 1980. x + 174 pp. f 11.50. MISCELLANEOUS J. ZELENY, The logic of Marx. Translated from the German by T. Carver. Oxford: Basil Blackwell, 1980. xcii + 247 pp. £12.50. FELIX KAUFMANN, The infinite in mathematics. Edited by Brian McGuinness. Introduction by E. Nagel. Translation from the German by Paul Foulkes. Dordrecht: Reidel, 1978. xvii + 235 pp. Dfl 85/$39.50 (cloth); Dfl 45/$19.95 (paper). PAMELA MCCORDUCK, Machines who think. San Francisco: W.H. Freeman and Company, 1979. xiv + 275 pp. $14.95. J. MITTELSTRASS (ed.), Enzyklopadie Philosophie und Wissenschaftstheorie Bd. 1 : A-G. Mannheim, Wien, Ziirich: Bibliographisches Institut, 1980. 835 pp. DM 128. (shrink)

Does action always arise out of desire? G. F. Schueler examines this hotly debated topic in philosophy of action and moral philosophy, arguing that once two senses of "desire" are distinguished - roughly, genuine desires and pro attitudes - apparently plausible explanations of action in terms of the agent's desires can be seen to be mistaken. Desire probes a fundamental issue in philosophy of mind, the nature of desires and how, if at all, they motivate and justify our actions. At (...) least since Hume argued that reason "is and of right ought to be the slave of the passions," many philosophers have held that desires play an essential role both in practical reason and in the explanation of intentional action. G. F. Schueler looks at contemporary accounts of both roles in various belief-desire models of reasons and explanation and argues that the usual belief-desire accounts need to be replaced. Schueler contends that the plausibility of the standard belief-desire accounts rests largely on a failure to distinguish "desires proper," like a craving for sushi, from so-called "pro attitudes," which may take the form of beliefs and other cognitive states as well as desires proper. Schueler's "deliberative model" of practical reasoning suggests a different view of the place of desire in practical reason and the explanation of action. He holds that we can arrive at an intention to act by weighing the relevant considerations and that these may not include desires proper at all. (shrink)

People act for reasons. That is how we understand ourselves. But what is it to act for a reason? This is what Fred Schueler investigates. He rejects the dominant view that the beliefs and desires that constitute our reasons for acting simply cause us to act as we do, and argues instead for a view centred on practical deliberation--our ability to evaluate the reasons we accept. Schueler's account of 'reasons explanations' emphasizes the relation between reasons and purposes, and the fact (...) that the reasons for an action are not always good reasons. (shrink)