Tissue engineering approaches to heart repair.

The heart is a large organ containing many cell types, each of which is necessary for normal function. Because of this, cardiac regenerative medicine presents many unique challenges. Because each of the many types of cells within the heart has unique physiological and electrophysiological characteristics, donor cells must be well matched to the area of the heart into which they are grafted to avoid mechanical dysfunction or arrhythmia.

ER stress signaling and neurodegeneration: At the intersection between Alzheimer's disease and Prion-related disorders.

Alzheimer's and Prion diseases are two neurodegenerative conditions sharing different pathophysiological characteristics. Disease symptoms are associated with the abnormal accumulation of protein aggregates, which are generated by the misfolding and oligomerization of specific proteins. Recent functional studies uncovered a key role of endoplasmic reticulum (ER) stress and the unfolded protein response (UPR) in the occurrence of synaptic dysfunction and neurodegeneration in Prion-related disorders and Alzheimer's disease.

Systematic review of the relationship between amyloid-β levels and measures of transgenic mouse cognitive deficit in Alzheimer's disease.

Amyloid-β (Aβ) is believed to directly affect memory and learning in Alzheimer's disease (AD). It is widely suggested that there is a relationship between Aβ40 and Aβ42 levels and cognitive performance. In order to explore the validity of this relationship, we performed a meta-analysis of 40 peer-reviewed, published AD transgenic mouse studies that quantitatively measured Aβ levels in brain tissue after assessing cognitive performance.

Gutsy moves in mice: cellular and molecular dynamics of endoderm morphogenesis.

Despite the importance of the gut and its accessory organs, our understanding of early endoderm development is still incomplete. Traditionally, endoderm has been difficult to study because of its small size and relative fragility. However, recent advances in live cell imaging technologies have dramatically expanded our understanding of this tissue, adding a new appreciation for the complex molecular and morphogenetic processes that mediate gut formation.

The use of mobile devices as assistive technology in resource-limited environments: access for learners with visual impairments in Kenya.

Purpose: In this paper, the authors explore the use of mobile devices as assistive technology for students with visual impairments in resource-limited environments. This paper provides initial data and analysis from an ongoing project in Kenya using tablet devices to provide access to education and independence for university students with visual impairments in Kenya.

Method: The project is a design-based research project in which we have developed and are refining a theoretically grounded intervention--a model for developing communities of practice to support the use of mobile technology as an assistive technology.

Results of a two-year pilot study of clinical outcome measures in collagen VI- and laminin alpha2-related congenital muscular dystrophies.

Potential therapies are currently under development for two congenital muscular dystrophy (CMD) subtypes: collagen VI-related muscular dystrophy (COL6-RD) and laminin alpha 2-related dystrophy (LAMA2-RD). However, appropriate clinical outcome measures to be used in clinical trials have not been validated in CMDs. We conducted a two-year pilot study to evaluate feasibility, reliability, and validity of various outcome measures, particularly the Motor Function Measure 32, in 33 subjects with COL6-RD and LAMA2-RD.

Safe and rapid disposition of low-to-intermediate risk patients presenting to the emergency department with chest pain: a 1-year high-volume single-center experience.

Background: Coronary CT angiography (CTA) is a powerful tool for the evaluation of chest pain in the emergency department (ED). Some debate persists regarding its cost-effectiveness in a low-to-intermediate risk population.

Objective: This study sought to evaluate the safety and cost-effectiveness of coronary CTA for low-to-intermediate risk patients presenting to the ED with chest pain in a closed-loop referral system.

Solitary Large Intestinal Diverticulitis in Leatherback Turtles (Dermochelys coriacea).

Leatherback sea turtles are globally distributed and endangered throughout their range. There are limited data available on disease in this species. Initial observations of solitary large intestinal diverticulitis in multiple leatherbacks led to a multi-institutional review of cases.

Mosaicism for dominant collagen 6 mutations as a cause for intrafamilial phenotypic variability.

Collagen 6-related dystrophies and myopathies (COL6-RD) are a group of disorders that form a wide phenotypic spectrum, ranging from severe Ullrich congenital muscular dystrophy, intermediate phenotypes, to the milder Bethlem myopathy. Both inter- and intrafamilial variable expressivity are commonly observed. We present clinical, immunohistochemical, and genetic data on four COL6-RD families with marked intergenerational phenotypic heterogeneity.

Management Strategies for Abdominal Bloating and Distension.

Bloating and distension are among the most common gastrointestinal complaints reported by patients with functional gut disorders and by the general population. These 2 complaints are also among the most prevalent of the severe symptoms reported by patients with irritable bowel syndrome. Nonetheless, only a limited number of published studies have specifically addressed bloating; it is infrequently studied as a primary endpoint, and what little systematic information exists has often been garnered from the assessment of secondary endpoints or the dissection of composite endpoints.

Observational study of spinal muscular atrophy type I and implications for clinical trials.

Objectives: Prospective cohort study to characterize the clinical features and course of spinal muscular atrophy type I (SMA-I).

Methods: Patients were enrolled at 3 study sites and followed for up to 36 months with serial clinical, motor function, laboratory, and electrophysiologic outcome assessments. Intervention was determined by published standard of care guidelines.

Placental Nkx2-5 and target gene expression in early-onset and severe preeclampsia.

Objective: Preeclampsia (PE) affects 2-8% of pregnancies worldwide and is a significant source of maternal and neonatal morbidity and mortality. However, the mechanisms underlying PE are poorly understood and major questions regarding etiology and risk factors remain to be addressed. Our objective was to examine whether abnormal expression of the cardiovascular developmental transcription factor, Nkx2-5, was associated with early onset and severe preeclampsia (EOSPE).

Limb girdle muscular dystrophy due to LAMA2 mutations: diagnostic difficulties due to associated peripheral neuropathy.

We report an eleven year old girl with early motor difficulties initially diagnosed with a peripheral neuropathy in another hospital based on abnormal electrophysiological findings. Our clinical assessment did not highlight obvious clinical features supporting a peripheral neuropathy but evidence of mild proximal weakness. Electrophysiological studies performed at our hospital revealed evidence of a sensorimotor demyelinating polyneuropathy with possible axonal involvement.

Testing models of the APC tumor suppressor/β-catenin interaction reshapes our view of the destruction complex in Wnt signaling.

The Wnt pathway is a conserved signal transduction pathway that contributes to normal development and adult homeostasis, but is also misregulated in human diseases such as cancer. The tumor suppressor adenomatous polyposis coli (APC) is an essential negative regulator of Wnt signaling inactivated in >80% of colorectal cancers. APC participates in a multiprotein "destruction complex" that targets the proto-oncogene β-catenin for ubiquitin-mediated proteolysis; however, the mechanistic role of APC in the destruction complex remains unknown.

Long-term behavior at foraging sites of adult female loggerhead sea turtles () from three Florida rookeries.

We used satellite telemetry to study behavior at foraging sites of 40 adult female loggerhead sea turtles () from three Florida (USA) rookeries. Foraging sites were located in four countries (USA, Mexico, the Bahamas, and Cuba). We were able to determine home range for 32 of the loggerheads.

Distinct tau prion strains propagate in cells and mice and define different tauopathies.

Prion-like propagation of tau aggregation might underlie the stereotyped progression of neurodegenerative tauopathies. True prions stably maintain unique conformations ("strains") in vivo that link structure to patterns of pathology. We now find that tau meets this criterion.

Facility for low-temperature spin-polarized-scanning tunneling microscopy studies of magnetic/spintronic materials prepared in situ by nitride molecular beam epitaxy.

Based on the interest in, as well as exciting outlook for, nitride semiconductor based structures with regard to electronic, optoelectronic, and spintronic applications, it is compelling to investigate these systems using the powerful technique of spin-polarized scanning tunneling microscopy (STM), a technique capable of achieving magnetic resolution down to the atomic scale. However, the delicate surfaces of these materials are easily corrupted by in-air transfers, making it unfeasible to study them in stand-alone ultra-high vacuum STM facilities. Therefore, we have carried out the development of a hybrid system including a nitrogen plasma assisted molecular beam epitaxy/pulsed laser epitaxy facility for sample growth combined with a low-temperature, spin-polarized scanning tunneling microscope system.

Whole-genome sequencing and the clinician: a tale of two cities.

Background: Clinicians are faced with unprecedented opportunities to identify the genetic aetiologies of hitherto molecularly uncharacterised conditions via the use of high-throughput sequencing. Access to genomic technology and resultant data is no longer limited to clinicians, geneticists and bioinformaticians, however; ongoing commercialisation gives patients themselves ever greater access to sequencing services. We report an increasingly common medical scenario by describing two neuromuscular patients--a mother and adult son--whose consumer access to whole-genome sequencing affected their diagnostic journey.

Fishery gear interactions from stranded bottlenose dolphins, Florida manatees and sea turtles in Florida, U.S.A.

Documenting the extent of fishery gear interactions is critical to wildlife conservation efforts, especially for reducing entanglements and ingestion. This study summarizes fishery gear interactions involving common bottlenose dolphins (Tursiops truncatus truncatus), Florida manatees (Trichechus manatus latirostris) and sea turtles: loggerhead (Caretta caretta), green turtle (Chelonia mydas), leatherback (Dermochelys coriacea), hawksbill (Eretmochelys imbricata), Kemp's ridley (Lepidochelys kempii), and olive ridley (Lepidochelys olivacea) stranding in Florida waters during 1997-2009. Fishery gear interactions for all species combined were 75.

Hydration in older adults: the contribution of bioelectrical impedance analysis.

The sensory and gastrointestinal changes that occur with ageing affect older adults' food and liquid intake. Any decreased liquid intake increases the risk for dehydration. This increased dehydration risk is compounded in older adults with dysphagia.

Pentyl-4-yn-VPA, a histone deacetylase inhibitor, ameliorates deficits in social behavior and cognition in a rodent model of autism spectrum disorders.

In utero exposure of rodents to valproic acid (VPA) has been proposed to induce an adult phenotype with behavioural characteristics reminiscent of those observed in autism spectrum disorder (ASD). Our previous studies have demonstrated the social cognition deficits observed in this model, a major core symptom of ASD, to be ameliorated following chronic administration of histone deacetylase (HDAC) inhibitors. Using this model, we now demonstrate pentyl-4-yn-VPA, an analogue of valproate and HDAC inhibitor, to significantly ameliorate deficits in social cognition as measured using the social approach avoidance paradigm as an indicator of social reciprocity and spatial learning to interrogate dorsal stream cognitive processing.

Yolk embolism associated with trauma in vitellogenic sea turtles in Florida (USA): a review of 11 cases.

Case information and postmortem examination findings are presented for 11 adult female sea turtles in reproductive form that died in Florida, USA. All had abundant, large vitellogenic follicles, and most were either gravid or had recently nested. Species included six loggerheads (Caretta caretta) and five green turtles (Chelonia mydas).

Computerized 3-dimensional localization of a video capsule in the abdominal cavity: validation by digital radiography.

Background: Wireless video capsule endoscopy allows the noninvasive visualization of the small intestine. Currently, capsules do not provide localization information while traversing the GI tract.

Objective: To report on the radiological validation of 3-dimensional localization software incorporated in a newly developed capsule.