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)

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)

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)

First published in 2002. Routledge is an imprint of Taylor & Francis, an informa company.

First published in 2002. Routledge is an imprint of Taylor & Francis, an informa company.

In the first part of this paper a logic is defined for propositions whose probability of being true may not be known. A speaker's beliefs about which propositions are true are still interesting in this case. The meaning of propositions is determined by the consequences of asserting them: in this logic there are debates which incur certain costs for the protagonists.The second part of the paper describes the mathematics of the resulting logic which displays several novel features.