Long-Term Follow-up Posthematopoietic Stem Cell Transplantation in a Japanese Patient with Type-VII Mucopolysaccharidosis

The effectiveness of hematopoietic stem cell transplantation (HSCT) for type-VII mucopolysaccharidosis (MPS VII, Sly syndrome) remains controversial, although recent studies have shown that it has a clinical impact. In 1998, Yamada et al. reported the first patient with MPS VII, who underwent HSCT at 12 years of age. Here, we report the results of a 22-year follow-up of that patient post-HSCT, who harbored the p.Ala619Val mutation associated with an attenuated phenotype. The purpose of this study was to evaluate changes in physical symptoms, the activity of daily living (ADL), and the intellectual status in the 34-year-old female MPS VII patient post-HSCT, and to prove the long-term effects of HSCT in MPS VII. Twenty-two years after HSCT, the β-glucuronidase activity in leukocytes remained at normal levels, and urinary glycosaminoglycan excretion was reduced and kept within normal levels. At present, she is capable of sustaining simple conversation, and her intellectual level is equivalent to that of a 6-year-old. She can walk alone and climb upstairs by holding onto a handrail, although she feels mild pain in the hip joint. The cervical vertebrae are fused with the occipital bone, causing dizziness and light-headedness when the neck is bent back. Overall, her clinical condition has been stabilized and kept well for long-term post-HSCT, indicating that HSCT is a therapeutic option for MPS VII

Formation and decay of cytochrome c peroxidase compound ES during aerobic redution with dithionite

AbstractStopped-flow and rapid scanning studies have clearly demonstrated that mixing of an oxygen-saturated solution of yeast cytochrome c peroxidase with sodium dithionite yields compound ES, indicating generation of H2O2. The formation of compound ES was most pronounced when [Na2S2O4/[O2] ≈ 1, and it reverted to the ferric form while standing. Even in the presence of an excess of dithionite ([Na2S2O4]/[O2] = 3.4) compound ES was formed immediately, but was soon replaced by the ferric form, followed by its final reduction to the ferrous state. The apparent first order rate constant for the decay of compound ES to the ferric form increased linearly with the square root of the dithionite concentration, thus involvement of SO−2 in that process being suggested

How valuable are chances?

Chance Neutrality is the thesis that, conditional on some proposition being true (or being false), its chance of being true should be a matter of practical indifference. The aim of this paper is to examine whether Chance Neutrality is a requirement of rationality. We prove that given Chance Neutrality, the Principal Principle entails a thesis called Linearity; the centrepiece of von Neumann and Morgenstern’s expected utility theory. With this in mind, we argue that the Principal Principle is a requirement of practical rationality but that Linearity is not; and hence, that Chance Neutrality is not rationally required

How valuable are chances?

Chance Neutrality is the thesis that, conditional on some proposition being true (or being false), its chance of being true should be a matter of practical indifference. The aim of this paper is to examine whether Chance Neutrality is a requirement of rationality. We prove that given Chance Neutrality, the Principal Principle entails a thesis called Linearity; the centrepiece of von Neumann and Morgenstern’s expected utility theory. With this in mind, we argue that the Principal Principle is a requirement of practical rationality but that Linearity is not; and hence, that Chance Neutrality is not rationally required

Fairness and risk attitudes

According to a common judgement, a social planner should often use a lottery to decide which of two people should receive a good. This judgement undermines one of the best-known arguments for utilitarianism, due to John C. Harsanyi, and more generally undermines axiomatic arguments for utilitarianism and similar views. In this paper we ask which combinations of views about (a) the social planner’s attitude to risk and inequality, and (b) the subjects’ attitudes to risk are consistent with the aforementioned judgement. We find that the class of combinations of views that can plausibly accommodate this judgement is quite limited. But one theory does better than others: the theory of chance-sensitive utility

Counterfactual Desirability

The desirability of what actually occurs is often influenced by what could have been. Preferences based on such value dependencies between actual and counterfactual outcomes generate a class of problems for orthodox decision theory, the best-known perhaps being the so-called Allais Paradox. In this paper we solve these problems by extending Richard Jeffrey's decision theory to counterfactual prospects, using a multidimensional possible-world semantics for conditionals, and showing that preferences that are sensitive to counterfactual considerations can still be desirability maximising. We end the paper by investigating the conditions necessary and sufficient for a desirability function to be an expected utility. It turns out that the additional conditions imply highly implausible epistemic principles