Lipid accumulation in dysferlin-deficient muscles.

Dysferlin is a membrane associated protein involved in vesicle trafficking and fusion. Defects in dysferlin result in limb-girdle muscular dystrophy type 2B and Miyoshi myopathy in humans and myopathy in A/J(dys-/-) and BLAJ mice, but the pathomechanism of the myopathy is not understood. Oil Red O staining showed many lipid droplets within the psoas and quadriceps muscles of dysferlin-deficient A/J(dys-/-) mice aged 8 and 12 months, and lipid droplets were also conspicuous within human myofibers from patients with dysferlinopathy (but not other myopathies).

Assessment of the potential relationship between egg quality and infectious bronchitis virus infection in Australian layer flocks.

Objective: This investigation aimed to determine if there was a relationship between the production of eggs with poor internal quality, as measured by poor Haugh units, by Australian layer flocks and the detection of infectious bronchitis virus (IBV) in the hens. Other risk factors including flock size, flock type, flock age, chicken breed and vaccination frequency were also assessed.

Methods: The study group comprised 17 flocks from 14 farms.

Mixed reality virtual pets to reduce childhood obesity.

Novel approaches are needed to reduce the high rates of childhood obesity in the developed world. While multifactorial in cause, a major factor is an increasingly sedentary lifestyle of children. Our research shows that a mixed reality system that is of interest to children can be a powerful motivator of healthy activity.

Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience.

Background: Absent pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary arteries and compression of the tracheobronchial tree and may lead to significant respiratory compromise. We describe the outcomes of surgical correction of absent pulmonary valve syndrome and risk factors for mortality and reoperation.

Methods: A review of 52 patients with absent pulmonary valve syndrome who underwent surgical correction between 1975 and 2013 was conducted.

The relationship between adult health and childhood maltreatment, as moderated by anger and ethnic background.

Childhood maltreatment, anger, and racial/ethnic background were examined in relation to physical health, psychological well-being, and blood pressure outcomes. This study used data from a diverse sample of African American, Latino, and Caucasian participants (N=198). Results from a series of multiple regressions indicated anger and total childhood maltreatment were robust predictors of poorer health.

Socioeconomic position across the lifecourse & allostatic load: data from the West of Scotland Twenty-07 cohort study.

Background: We examined how socioeconomic position (SEP) across the lifecourse (three critical periods, social mobility and accumulated over time) is associated with allostatic load (a measure of cumulative physiological burden).

Methods: Data are from the West of Scotland Twenty-07 Study, with respondents aged 35 (n = 740), 55 (n = 817) and 75 (n = 483). SEP measures representing childhood, the transition to adulthood and adulthood SEP were used.

Posterior fossa and spinal gangliogliomas form two distinct clinicopathologic and molecular subgroups.

Background: Gangliogliomas are low-grade glioneuronal tumors of the central nervous system and the commonest cause of chronic intractable epilepsy. Most gangliogliomas (>70%) arise in the temporal lobe, and infratentorial tumors account for less than 10%. Posterior fossa gangliogliomas can have the features of a classic supratentorial tumor or a pilocytic astrocytoma with focal gangliocytic differentiation, and this observation led to the hypothesis tested in this study - gangliogliomas of the posterior fossa and spinal cord consist of two morphologic types that can be distinguished by specific genetic alterations.

Do mismatches between pre- and post-natal environments influence adult physiological functioning?

Purpose: Mismatches between pre- and post-natal environments have implications for disease in adulthood. However, less is known about how this mismatch can affect physiological systems more generally, especially at younger ages. We hypothesised that mismatches between pre- and post-natal environments, as measured by the measures of birthweight and adult leg length, would be associated with poorer biomarker levels across five key physiological systems in young adults.

Evaluation of neonatal peripherally inserted central catheter tip movement in a consistent upper extremity position.

Objective: To compare the effect of standardized upper extremity position versus varied upper extremity positions on neonatal peripherally inserted central catheter (PICC) tip placement and movement. Secondary outcomes assessed were compliance with education, implementation, and complication rates.

Study Design: Tip movement was analyzed between 136 post-PICC insertion x-ray pairs from 72 infants in the 6 months before and after standardization of upper extremity position.

An international, multicentre survey of β-lactam antibiotic therapeutic drug monitoring practice in intensive care units.

Objectives: Emerging evidence supports the use of therapeutic drug monitoring (TDM) of β-lactams for intensive care unit (ICU) patients to optimize drug exposure, although limited detail is available on how sites run this service in practice. This multicentre survey study was performed to describe the various approaches used for β-lactam TDM in ICUs.

Methods: A questionnaire survey was developed to describe various aspects relating to the conduct of β-lactam TDM in an ICU setting.

Gender and telomere length: systematic review and meta-analysis.

Background: It is widely believed that females have longer telomeres than males, although results from studies have been contradictory.

Methods: We carried out a systematic review and meta-analyses to test the hypothesis that in humans, females have longer telomeres than males and that this association becomes stronger with increasing age. Searches were conducted in EMBASE and MEDLINE (by November 2009) and additional datasets were obtained from study investigators.

G-protein coupled receptor expression patterns delineate medulloblastoma subgroups.

Background: Medulloblastoma is the most common malignant brain tumor in children. Genetic profiling has identified four principle tumor subgroups; each subgroup is characterized by different initiating mutations, genetic and clinical profiles, and prognoses. The two most well-defined subgroups are caused by overactive signaling in the WNT and SHH mitogenic pathways; less is understood about Groups 3 and 4 medulloblastoma.

Glioma surgical aspirate: a viable source of tumor tissue for experimental research.

Brain cancer research has been hampered by a paucity of viable clinical tissue of sufficient quality and quantity for experimental research. This has driven researchers to rely heavily on long term cultured cells which no longer represent the cancers from which they were derived. Resection of brain tumors, particularly at the interface between normal and tumorigenic tissue, can be carried out using an ultrasonic surgical aspirator (CUSA) that deposits liquid (blood and irrigation fluid) and resected tissue into a sterile bottle for disposal.

Giant axonal neuropathy diagnosed on skin biopsy.

Evaluation of hereditary axonal neuropathy in childhood is complex. Often, the child has to be subjected to general anaesthesia for a nerve biopsy to guide further genetic testing, which may or may not be readily available. We describe a toddler with clinical features suggesting giant axonal neuropathy (GAN), whose diagnosis was confirmed by minimally invasive skin biopsy and corroborated by the finding of compound heterozygous mutations involving the GAN gene, including a novel interstitial microdeletion at 16q23.

Vasopressin-induced constriction of the isolated rat occipital artery is segment dependent.

Background: Circulating factors delivered to the nodose ganglion (NG) by the occipital artery (OA) have been shown to affect vagal afferent activity, and thus the contractile state of the OA may influence blood flow to the NG.

Methods: OA were isolated and bisected into proximal and distal segments relative to the external carotid artery.

Results: Bisection highlighted stark differences between maximal contractile responses and OA sensitivity.

Oral intensity: reducing non-ventilator-associated hospital-acquired pneumonia in care-dependent, neurologically impaired patients.

Introduction: The purpose of this point-of-care study was to test the efficacy of a prevention-based oral care protocol in reducing non-ventilator-associated hospital-acquired pneumonia in a neurosurgical population outside the critical care environment. The researchers hypothesized that an enhanced oral care protocol would decrease the incidence of pneumonia.

Methods: This quasi-experimental, comparative study took place on an acute neurosurgical unit at a tertiary care trauma hospital in Western Canada.

Long-term outcomes of dilated cardiomyopathy diagnosed during childhood: results from a national population-based study of childhood cardiomyopathy.

Background: Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy.

Methods And Results: The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings.

Absence of neurotoxicity with perineural injection of ultrasound gels: assessment using an animal model.

Background: Ultrasound gels may contain propylene glycol and glycerol, which are neurotoxic in high concentrations. If the needle passes through gel during regional anesthesia, gel may be injected near the nerve. It is unknown if this practice poses a risk for neurotoxicity.

Balancing theory and practice in respondent-driven sampling: a case study of innovations developed to overcome recruitment challenges.

Introduction: Respondent-driven sampling (RDS) offers a recruitment strategy for hard-to-reach populations. However, RDS faces logistical and theoretical challenges that threaten efficiency and validity in settings worldwide. We present innovative adaptations to conventional RDS to overcome barriers encountered in recruiting a large, representative sample of men who have sex with men (MSM) who travel internationally.

Disparate effects of LPS infusion and carbohydrate overload on inflammatory gene expression in equine laminae.

Although clinical evidence of endotoxemia has been associated with the development of acute laminitis in hospitalized horses with gastrointestinal diseases and endotoxins have been detected in the circulation of horses with experimentally-induced laminitis, it is unclear what role, if any, endotoxins have play the pathogenesis of the disease. Therefore, in the present study we compared the effects of endotoxin infusion to that of intra-gastric administration of mixed carbohydrate (CHO) on clinical signs of laminitis, plasma concentrations of TNF-α and IL-10, and laminar tissue expression of 20 genes associated with inflammation. Horses were divided into 4 groups: Control (water placebo, n=7), endotoxin infusion (LPS, n=6), CHO/Developmental (30% decrease in central venous pressure, n=6) and CHO/Lame (Obel grade I laminitis, n=7).

Merely opting out of a public good is moralized: an error management approach to cooperation.

People regularly free ride on collective benefits, consuming them without contributing to their creation. In response, free riders are often moralized, becoming targets of negative moral judgments, anger, ostracism, or punishment. Moralization can change free riders' behavior (e.

Current and emerging pharmacotherapeutic options for smoking cessation.

Tobacco smoking remains the single most preventable cause of morbidity and mortality in developed countries and poses a significant threat across developing countries where tobacco use prevalence is increasing. Nicotine dependence is a chronic disease often requiring multiple attempts to quit; repeated interventions with pharmacotherapeutic aids have become more popular as part of cessation therapies. First-line medications of known efficacy in the general population include varenicline tartrate, bupropion hydrochloride, nicotine replacement therapy products, or a combination thereof.