Evidence‐based guidelines support integrated disease management as the optimal model of haemophilia care

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/122450/1/hae12997_am.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/122450/2/hae12997.pd

Evidence‐based guidelines support integrated disease management as the optimal model of haemophilia care

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/122450/1/hae12997_am.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/122450/2/hae12997.pd

MCFD2, a second gene defective in combined factor V/factor VIII deficiency, encodes a novel ER protein that interacts with LMAN1 (ERGIC‐53) to form a cofactor‐specific sorting receptor

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106096/1/jth03926.pd

Biological mechanisms underlying inter‐individual variation in factor VIII clearance in haemophilia

Previous studies have highlighted marked inter‐individual variations in factor VIII (FVIII) clearance between patients with haemophilia (PWH). The half‐life of infused FVIII has been reported to vary from as little as 5.3 hours in some adult PWH, up to as long as 28.8 hours in other individuals. These differences in clearance kinetics have been consistently observed using a number of different plasma‐derived and recombinant FVIII products. Furthermore, recent studies have demonstrated that half‐life for extended half‐life (EHL‐) FVIII products also demonstrates significant inter‐patient variation. Since time spent with FVIII trough levels <1% has been shown to be associated with increased bleeding risk in PWH on prophylaxis therapy, this variability in FVIII clearance clearly has major clinical significance. Recent studies have provided significant novel insights into the cellular basis underlying FVIII clearance pathways. In addition, accumulating data have shown that endogenous plasma VWF levels, ABO blood group and age, all play important roles in regulating FVIII half‐life in PWH. Indeed, multiple regression analysis suggests that together these factors account for approximately 34% of the total inter‐individual variation in FVIII clearance observed between subjects with severe haemophilia A. In this review, we consider these and other putative modulators of FVIII half‐life, and discuss the biological mechanisms through which these factors impact upon FVIII clearance in vivo.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/156160/2/hae14078.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/156160/1/hae14078_am.pd

Genetic modifications that increase factor VIII secretion in vitro and in vivo

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106156/1/jth03924.pd

Advantage of recombinant von Willebrand factor for peri‐operative management in paediatric acquired von Willebrand syndrome

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/144273/1/hae13436_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/144273/2/hae13436.pd

The DRIFT Dark Matter Experiments

The current status of the DRIFT (Directional Recoil Identification From Tracks) experiment at Boulby Mine is presented, including the latest limits on the WIMP spin-dependent cross-section from 1.5 kg days of running with a mixture of CS2 and CF4. Planned upgrades to DRIFT IId are detailed, along with ongoing work towards DRIFT III, which aims to be the world's first 10 m3-scale directional Dark Matter detector.Comment: Proceedings of the 3rd International conference on Directional Detection of Dark Matter (CYGNUS 2011), Aussois, France, 8-10 June 201

Microdevices for extensional rheometry of low viscosity elastic liquids : a review

Extensional flows and the underlying stability/instability mechanisms are of extreme relevance to the efficient operation of inkjet printing, coating processes and drug delivery systems, as well as for the generation of micro droplets. The development of an extensional rheometer to characterize the extensional properties of low viscosity fluids has therefore stimulated great interest of researchers, particularly in the last decade. Microfluidics has proven to be an extraordinary working platform and different configurations of potential extensional microrheometers have been proposed. In this review, we present an overview of several successful designs, together with a critical assessment of their capabilities and limitations

Keeping secrets from parents: Advantages and disadvantages of secrecy in adolescence.

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