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The current status of prophylactic replacement therapy in children and adults with haemophilia.

Initiating prophylactic treatment at an early age is considered to be the optimal form of therapy for a child with haemophilia A or B. The pioneering long term experiences of prophylactic treatment from Sweden and The Netherlands demonstrated the benefit of prophylaxis in retrospective and observational studies. Decades later, these benefits were confirmed in a randomized controlled study in USA.

Surface and core contribution to 1/f-noise in InAs nanowire metal-oxide-semiconductor field-effect transistors

By measuring 1/f-noise in wrap-gated InAs nanowire metal-oxide-semiconductor field-effect transistors with transport dominating, controllably, in either an inner, core channel, or an outer, surface channel, it is possible to accurately evaluate the material quality related Hooge-parameter, alpha(H), with reduced interference from the surface properties. The devices show low values of alpha(H) simi

Comparing top-down and bottom-up costing approaches for economic evaluation within social welfare.

This study compares two approaches to the estimation of social welfare intervention costs: one "top-down" and the other "bottom-up" for a group of social welfare clients with severe problem behavior participating in a randomized trial. Intervention costs ranging over a two-year period were compared by intervention category (foster care placement, institutional placement, mentorship services, indiv

Female Rule in the Indian Ocean World (1300-1900)

An outstanding feature of the early modern Indian Ocean World is the large number of women who exercised formal sovereign political power. Based on a systematic survey of 277 queens regnant in the Indian Ocean World from the fourteenth to the nineteenth century, this article discusses four possible explanations for the relative frequency of female rule: religion, trade, political stability, and ge

An Efficient Optimization Framework for Multi-Region Segmentation based on Lagrangian Duality

We introduce a multi-region model for simultaneous segmentation of medical images. In contrast to many other models, geometric constraints such as inclusion and exclusion between the regions are enforced, which makes it possible to correctly segment different regions even if the intensity distributions are identical. We efficiently optimize the model using a combination of graph cuts and Lagrangia

RNA and DNA Association to Zwitterionic and Charged Monolayers at the Air-Liquid Interface

The objective of this work is to establish under which conditions short RNA molecules (similar to miRNA) associate with zwitterionic phospholipids and how this differs from the association with cationic surfactants. We study how the base pairing (i.e., single stranded versus double stranded nucleic acids) and the length of the nucleic acid and the charge of the lipid/surfactant monolayer affect th

Genome-Wide Association Meta-Analysis of Cortical Bone Mineral Density Unravels Allelic Heterogeneity at the RANKL Locus and Potential Pleiotropic Effects on Bone

Previous genome-wide association (GWA) studies have identified SNPs associated with areal bone mineral density (aBMD). However, this measure is influenced by several different skeletal parameters, such as periosteal expansion, cortical bone mineral density (BMDC) cortical thickness, trabecular number, and trabecular thickness, which may be under distinct biological and genetic control. We have car

Modeling the anaerobic digestion of cane-molasses vinasse: Extension of the Anaerobic Digestion Model No. 1 (ADM1) with sulfate reduction for a very high strength and sulfate rich wastewater.

This research presents the modeling of the anaerobic digestion of cane-molasses vinasse, hereby extending the Anaerobic Digestion Model No. 1 with sulfate reduction for a very high strength and sulfate rich wastewater. Based on a sensitivity analysis, four parameters of the original ADM1 and all sulfate reduction parameters were calibrated. Although some deviations were observed between model pred

Modes of learning and entrepreneurial knowledge

In this study, we examine how entrepreneurs differ in their modes of learning and whether variations in modes of learning have any influence on their possession of entrepreneurial knowledge that increase their ability to recognise and exploit new business opportunities. Based on statistical analysis of 291 entrepreneurs we find that a learning mode that favours exploration is positively associated

Ribosomal DNA Organization Patterns within the Dinoflagellate Genus Alexandrium as Revealed by FISH: Life Cycle and Evolutionary Implications.

Dinoflagellates are a group of protists whose genome differs from that of other eukaryotes in terms of size (contains up to 250pg per haploid cell), base composition, chromosomal organization, and gene expression. But rDNA gene mapping of the active nucleolus in this unusual eukaryotic genome has not been carried out thus far. Here we used FISH in dinoflagellate species belonging to the genus Alex

Genome-wide linkage analysis in families with infantile hypertrophic pyloric stenosis indicates novel susceptibility loci

Infantile hypertrophic pyloric stenosis (IHPS) is a common cause of upper gastrointestinal obstruction during infancy. A multifactorial background of the disease is well established. Multiple susceptibility loci including the neuronal nitric oxide synthase (NOS1) gene have previously been linked to IHPS, but contradictory results of linkage studies in different materials indicate genetic heterogen

On the complexation of whey proteins

Milk proteins have a rich diversity of physical chemistry and biodegradable properties which makes them appealing for different food and pharmaceutical applications. Theoretical coarse grained models and numerical simulations were employed here in order to gain novel insight into the understanding of the fundamental mechanisms of the process of milk proteins complexation in a diversity of environm

Diffraction of slow neutrons by holographic SiO2 nanoparticle-polymer composite gratings

Diffraction experiments with holographic gratings recorded in SiO2 nanoparticle-polymer composites have been carried out with slow neutrons. The influence of parameters such as nanoparticle concentration, grating thickness, and grating spacing on the neutron-optical properties of such materials has been tested. Decay of the grating structure along the sample depth due to disturbance of the recordi

A stable liquid-liquid extraction system for clavulanic acid using polymer-based aqueous two-phase systems

The partitioning of Clavulanic Acid (CA) in a novel inexpensive and stable aqueous two-phase system (ATPS) composed by poly(ethylene glycol) (PEG) and sodium polyacrylate (NaPA) has been studied. The aqueous two-phase systems are formed by mixing both polymers with a salt (NaCl or Na2SO4) and an aqueous solution of CA. The stability of CA on the presence of both polymers was investigated and it wa

Fluorescent Boronic Acid Polymer Grafted on Silica Particles for Affinity Separation of Saccharides.

Boronic acid affinity gels are important for effective separation of biological active cis-diols, and are finding applications both in biotech industry and in biomedical research areas. To increase the efficacy of boronate affinity separation, it is interesting to introduce repeating boronic acid units in flexible polymer chains attached on solid materials. In this work, we synthesize polymer brus

Predicting Acidification Recovery at the Hubbard Brook Experimental Forest, New Hampshire: Evaluation of Four Models

The performance and prediction uncertainty (owing to parameter and structural uncertainties) of four dynamic watershed acidification models (MAGIC, PnET-BGC, SAFE, and VSD) were assessed by systematically applying them to data from the Hubbard Brook Experimental Forest (HBEF), New Hampshire, where long-term records of precipitation and stream chemistry were available. In order to facilitate system