Clinical practice of personalized prophylaxis in hemophilia: Illustrations of experiences and benefits from two continents.

These cases of people with hemophilia (PWH) illustrate the importance of understanding the patient's expectations and desires and adapting treatment to meet these needs, in addition to traditional clinical targets. Population PK modeling and FVIII products with improved PK profiles provide the opportunity to individualize care and improve long-term outcomes.

Population genomic analyses reveal a highly differentiated and endangered genetic cluster of northern goshawks () in Haida Gwaii.

Accurate knowledge of geographic ranges and genetic relationships among populations is important when managing a species or population of conservation concern. Along the western coast of Canada, a subspecies of the northern goshawk () is legally designated as Threatened. The range and distinctness of this form, in comparison with the broadly distributed North American subspecies (), is unclear.

Conservation of genetic diversity hotspots of the high-valued relic yellowhorn () considering climate change predictions.

Genetic structure and major climate factors may contribute to the distribution of genetic diversity of a highly valued oil tree species (yellowhorn). Long-term over utilization along with climate change is affecting the viability of yellowhorn wild populations. To preserve the species known and unknown valuable gene pools, the identification of genetic diversity "hotspots" is a prerequisite for their consideration as in situ conservation high priority.

Patterns and biases in an Arctic herbarium specimen collection: Implications for phenological research.

Premise Of The Study: Herbarium specimens are increasingly used in phenological studies. However, natural history collections can have biases that influence the analysis of phenological events. Arctic environments, where remoteness and cold climate govern collection logistics, may give rise to unique or pronounced biases.

Systematic Incorporation of Sex-Specific Information Into Clinical Practice Guidelines for the Management of ST -Segment-Elevation Myocardial Infarction: Feasibility and Outcomes.

Background Clinical practice guideline ( CPG ) developers have yet to endorse a consistent and systematic approach for considering sex-specific cardiovascular information in CPG s. This article describes an initiative led by the Canadian Cardiovascular Society to determine the feasibility and outcomes of a structured process for considering sex in a CPG for the management of ST-segment-elevation myocardial infarction. Methods and Results A sex and gender champion was appointed to the guideline development committee.

Efficacy and Safety of Bempedoic Acid in Patients With Hypercholesterolemia and Statin Intolerance.

Background Inability to tolerate statins because of muscle symptoms contributes to uncontrolled cholesterol levels and insufficient cardiovascular risk reduction. Bempedoic acid, a prodrug that is activated by a hepatic enzyme not present in skeletal muscle, inhibits ATP -citrate lyase, an enzyme upstream of β-hydroxy β-methylglutaryl-coenzyme A reductase in the cholesterol biosynthesis pathway. Methods and Results The phase 3, double-blind, placebo-controlled CLEAR (Cholesterol Lowering via Bempedoic acid, an ACL-Inhibiting Regimen) Serenity study randomized 345 patients with hypercholesterolemia and a history of intolerance to at least 2 statins (1 at the lowest available dose) 2:1 to bempedoic acid 180 mg or placebo once daily for 24 weeks.

Predictors of new graduate nurses' health over the first 4 years of practice.

Aim: To examine predictors of Canadian new graduate nurses' health outcomes over 1 year.

Design: A time-lagged mail survey was conducted.

Method: New graduate nurses across Canada ( = 406) responded to a mail survey at two time points: November 2012-March 2013 (Time 1) and May-July 2014 (Time 2).

Longitudinal advanced MRI case report of white matter radiation necrosis.

Radiation necrosis mostly occurs in and near the radiation field. We used magnetic resonance imaging to study radiation-induced necrosis of atypical onset, severity, and extent following stereotactic radiosurgery for a symptomatic arteriovenous malformation. Susceptibility-sensitive imaging, T-relaxation, myelin water imaging, and magnetic resonance spectroscopy were acquired three times up to 52 months postradiosurgery.

A roadmap for urban evolutionary ecology.

Urban ecosystems are rapidly expanding throughout the world, but how urban growth affects the evolutionary ecology of species living in urban areas remains largely unknown. Urban ecology has advanced our understanding of how the development of cities and towns change environmental conditions and alter ecological processes and patterns. However, despite decades of research in urban ecology, the extent to which urbanization influences evolutionary and eco-evolutionary change has received little attention.

Mammal extinctions and the increasing isolation of humans on the tree of life.

A sixth great mass extinction is ongoing due to the direct and indirect effects of human pressures. However, not all lineages are affected equally. From an anthropocentric perspective, it is often purported that humans hold a unique place on Earth.

Cerebral White Matter Hyperintensities, Kidney Function Decline, and Recurrent Stroke After Intensive Blood Pressure Lowering: Results From the Secondary Prevention of Small Subcortical Strokes ( SPS 3) Trial.

Background We aimed to determine whether cerebral white matter hyperintensities ( WMHs ) can distinguish stroke survivors susceptible to rapid kidney function decline from intensive blood pressure ( BP ) lowering. Methods and Results The SPS3 (Secondary Prevention of Small Subcortical Strokes) trial randomized participants with recent lacunar stroke to systolic BP targets of 130 to 149 and <130 mm Hg. We included 2454 participants with WMH measured by clinical magnetic resonance imaging at baseline and serum creatinine measured during follow-up.

Sensorimotor plasticity after spinal cord injury: a longitudinal and translational study.

Objective: The objective was to track and compare the progression of neuroplastic changes in a large animal model and humans with spinal cord injury.

Methods: A total of 37 individuals with acute traumatic spinal cord injury were followed over time (1, 3, 6, and 12 months post-injury) with repeated neurophysiological assessments. Somatosensory and motor evoked potentials were recorded in the upper extremities above the level of injury.

The genomics of domestication special issue editorial.

Domestication has been of major interest to biologists for centuries, whether for creating new plants and animal types or more formally exploring the principles of evolution. Such studies have long used combinations of phenotypic and genetic evidence. Recently, the advent of a large number of genomes and genomic tools across a wide array of domesticated plant and animal species has reinvigorated the study of domestication.

Association Between Wait Time for Transcatheter Aortic Valve Replacement and Early Postprocedural Outcomes.

Background Rapid growth in transcatheter aortic valve replacement ( TAVR ) demand has translated to inadequate access, reflected by prolonged wait times. Increasing wait times are associated with important adverse outcomes while on the wait-list; however, it is unknown if prolonged wait times influence postprocedural outcomes. Our objective was to determine the association between TAVR wait times and postprocedural outcomes.

Maladaptive secondary sexual characteristics reduce the reproductive success of hybrids between native and non-native salmonids.

Human-mediated hybridization between introduced and native species is one of the most serious threats to native taxa. Although field studies have attempted to quantify the relative fitness or reproductive success of parental species and their hybrids only a few studies have unraveled the factors determining the fitness of hybrids. Here, we hypothesized that maladaptive secondary sexual characteristics may reduce fitness of hybrids between two fish species.

Association Between Driving Distance From Nearest Fire Station and Survival of Out-of-Hospital Cardiac Arrest.

Background Firefighter first responders dispatched in parallel with emergency medical services ( EMS ) personnel for out-of-hospital cardiac arrests ( OHCA ) can provide early defibrillation to improve survival. We examined whether survival following first responder defibrillation differed according to driving distance from nearest fire station to OHCA site. Methods and Results From the CARES (Cardiac Arrest Registry to Enhance Survival) registry, we identified non- EMS witnessed OHCA s of presumed cardiac cause from 2010 to 2014 in Durham, Mecklenburg, and Wake counties, North Carolina.

Large-scale molecular diet analysis in a generalist marine mammal reveals male preference for prey of conservation concern.

Sex-specific diet information is important in the determination of predator impacts on prey populations. Unfortunately, the diet of males and females can be difficult to describe, particularly when they are marine predators. We combined two molecular techniques to describe haul-out use and prey preferences of male and female harbor seals () from Comox and Cowichan Bay (Canada) during 2012-2013.

Altered tryptophan metabolism is associated with pediatric multiple sclerosis risk and course.

Objective: To determine if altered tryptophan (Trp) metabolism is associated with MS risk or disease severity in children.

Methods: Participants with pediatric-onset MS and clinically isolated syndrome (CIS) within 4 years of disease onset and healthy controls underwent collection of serum. Longitudinal disability and processing speed measures and relapse data were collected in cases.

Physiological and genomic signatures of evolutionary thermal adaptation in redband trout from extreme climates.

Temperature is a master environmental factor that limits the geographical distribution of species, especially in ectotherms. To address challenges in biodiversity conservation under ongoing climate change, it is essential to characterize relevant functional limitations and adaptive genomic content at population and species levels. Here, we present evidence for adaptive divergence in cardiac function and genomic regions in redband trout () populations from desert and montane streams.

Functional MRI on executive functioning in aging and dementia: A scoping review of cognitive tasks.

Cognitive decline with aging and dementia is especially poignant with regard to the executive functioning that is necessary for activities of daily independent living. The relationship between age-related neurodegeneration in the prefrontal cortex and executive functioning has been uniquely investigated using task-phase functional magnetic resonance imaging (fMRI) to detect brain activity in response to stimuli; however, a comprehensive list of task designs that have been implemented to task-phase fMRI is absent in the literature. The purpose of this review was to recognize what methods have been used to study executive functions with aging and dementia in fMRI tasks, and to describe and categorize them.

Guide design in virtual planning for scapular tip free flap reconstruction.

Background: Virtual surgical planning (VSP), intraoperative cutting guides and stereolithographic models, provides the head and neck reconstructive surgeon with powerful tools for complex reconstruction planning. Despite its use in fibular osteocutaneous reconstruction, application to the scapular tip has not been as widely reported.

Methods: From 2013 to 2014, four cases of either mandibular or maxillary reconstruction were completed with the scapular tip osseous free flap.

A young woman with steroid-responsive, IgG4-positive plasma cell-enriched cystic lymphangioma and chylous ascites.

Lymphangiomas are benign tumors of the lymphatic vessels, which can be inflammatory and occasionally steroid-responsive. IgG4-related disease (IgG4-RD) is a recently defined fibro-inflammatory condition. We describe a novel association between reactive IgG4+ plasma cells and cystic lymphangioma in a young woman who had a dramatic clinical response to steroids.

The relationship between d-beta-hydroxybutyrate blood concentrations and seizure control in children treated with the ketogenic diet for medically intractable epilepsy.

Objective: The ketogenic diet (KD) is a proven treatment for drug-resistant (DR) seizures in children and adolescents. However, the relationship between seizure control and the most commonly measured metabolite of the diet, the ketone body d-beta-hydroxybutyrate (D-BHB), is controversial. This study was performed to clarify the relationship because specific ketone bodies may be useful as biomarkers of diet efficacy.

Quantifying the Contribution to Uncertainty in Mortality Attributed to Household, Ambient, and Joint Exposure to PM From Residential Solid Fuel Use.

While there have been substantial efforts to quantify the health burden of exposure to PM from solid fuel use (SFU), the sensitivity of mortality estimates to uncertainties in input parameters has not been quantified. Moreover, previous studies separate mortality from household and ambient air pollution. In this study, we develop a new estimate of mortality attributable to SFU due to the joint exposure from household and ambient PM pollution and perform a variance-based sensitivity analysis on mortality attributable to SFU.