Enzyme replacement is a potential therapy for mucopolysaccharidosis I (MPS I), a lysosomal storage disorder caused by alpha-L-iduronidase deficiency. Previous work showed improvement in the tissues of MPS I dogs treated intravenously for 3 months with recombinant human alpha-L-iduronidase (25,000 units or approximately 0.1 mg/kg/week). We have now treated an MPS I-affected dog for 13 months to ass

BACKGROUND: During a recent armed conflict in Guinea-Bissau, we observed a marked decline in the case fatality among hospitalized children at the only paediatric department in the country. AIM: To analyse the causes behind the observed fall in case fatality. MATERIAL: All children hospitalized at the only paediatric department in the capital of Guinea-Bissau. The war cohort comprised all children

Searches for HZ production with the Higgs boson decaying into an invisible final state were performed using the data collected by the DELPHI experiment at centre-of-mass energies between 188 GeV and 209 GeV. Both hadronic and leptonic final states of the Z boson were analysed. In addition to the search for a heavy Higgs boson, a dedicated search for a light Higgs boson down to 40 GeV/c(2) was perf

A beamline for macromolecular crystallography is under construction at the Swedish synchrotron light source MAX-lab at Lund University in a collaborative effort between Denmark and Sweden. Of the 7 mrad horizontal wiggler fan emitted from the new superconducting multipole wiggler, the central 2 mrad will be used and split in three parts. The central 1 mrad will be used for a tunable station optimi

The aim of this study was to estimate costs of treating venous leg ulcers in Sweden and the United Kingdom during 1 year and to quantify costs in different health states, The costs of treating four different types of venous leg ulcers were estimated for 52 weeks by a stochastic health economic model, which simulated resource use data obtained from prospectively collected patient data, expert panel

It is hard to defend the view that biotic communities represent a simple and predictable response to the abiotic environment. Biota and the abiotic environment interact, and the environment of an individual certainly includes its neighbors and visitors in the community. The complexity of community assembly calls forth a quest for general principles, yet current results and theories on assembly rul

Background: The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative disorders characterized by accumulation of autofluorescent material in many tissues, especially in neurons. Mutations in the CLN8 gene, encoding an endoplasmic reticulum ( ER) transmembrane protein of unknown function, underlie NCL phenotypes in humans and mice. The human phenotype is characterized by

The article tries to give a solution to a key problem for modern religious man and modern theology: what kind of reality is at stake in religious language and religious belief? Questions of how reality is related to concepts and social contexts are discussed particularly with respect to children's make-believe play, and a distinction is introduced between reality as conceptualized by us, as constr

Protein kinase B (PKB) is involved in signaling to a multitude of important cellular events and is activated by insulin and growth factors, including insulin-like growth factor I (IGF-I). We show here expression of PKB in pancreatic islets and in the beta cell lines HIT-T15, INS-1, and RINm5F. Expression of PKB mRNA and the presence of PKB isoforms (alpha, beta, and gamma) were assessed by Norther

Many issues in computational vision can be understood from the interplay between camera geometry and the structure of images and objects. Typically, the image structure is available and the goal is to reconstruct object structure and camera geometry. This is often difficult due to the complex interdependence between these three entities. The theme of this thesis is to use invariants to solve these

Helicobacter species are fastidious bacterial pathogens that are difficult to culture by standard methods. A PCR-denaturing gradient gel electrophoresis (PCR-DGGE) technique for detection and identification of different Helicobacter species was developed and evaluated. The method involves PCR detection of Helicobacter DNA by genus-specific primers that target 16S rDNA and subsequent differentiatio

The immunodeficiency, centromeric region instability, facial anomalies (ICF) syndrome is a rare autosomal recessive disease. Usually, it is caused by mutations in the DNA methyltransferase 3B gene, which result in decreased methylation of satellite DNA in the juxtacentromeric heterochromatin at 1qh, 16qh, and 9qh. Satellite II-rich 1qh and 16qh display high frequencies of abnormalities in mitogen-

Hans Rott has argued, most recently in his book Change, Choice and Inference, that certain formal correspondences between belief revision and rational choice have important philosophical implications, claiming that the former strongly indicate the unity of practical and theoretical reason as well as the primacy of practical reason. In this paper, I confront Rott's argument with three serious chall