Williams-Beuren Syndrome and Epilepsy: A Retrospective Analysis of 589 Patients.

Although many reports have described the characteristics of Williams-Beuren syndrome, few specifically analyzed epilepsy in patients with Williams-Beuren syndrome. In this retrospective study, we map the prevalence, types, and prognosis of epileptic seizures in a large cohort of 589 patients with Williams-Beuren syndrome, as well as associations between deletions of the membrane-associated guanylate kinase inverted-2-gene (2 gene), which is associated with infantile spasms (IS), and epilepsy in patients with Williams-Beuren syndrome.Our findings indicate that the incidence of epilepsy in patients with Williams-Beuren syndrome is approximately 1.

ATP Synthase K- and H-Fluxes Drive ATP Synthesis and Enable Mitochondrial K-"Uniporter" Function: I. Characterization of Ion Fluxes.

ATP synthase (FF) synthesizes daily our body's weight in ATP, whose production-rate can be transiently increased several-fold to meet changes in energy utilization. Using purified mammalian FF-reconstituted proteoliposomes and isolated mitochondria, we show FF can utilize both ΔΨ-driven H- and K-transport to synthesize ATP under physiological pH = 7.2 and K = 140 mEq/L conditions.

ATP Synthase K- and H-fluxes Drive ATP Synthesis and Enable Mitochondrial K-"Uniporter" Function: II. Ion and ATP Synthase Flux Regulation.

We demonstrated that ATP synthase serves the of a primary mitochondrial K "uniporter," i.e., the primary way for K to enter mitochondria.

Maternal pre-pregnancy weight status modifies the influence of PUFAs and inflammatory biomarkers in breastmilk on infant growth.

Background: Human breastmilk contains pro- and anti-inflammatory compounds and hormones that can influence infant growth. However, little is known about the specific interrelationships between these compounds and whether their effects on infant growth may be influenced by pre-pregnancy weight status.

Objective: The purpose of this novel, prospective cohort study was to assess the interrelationships between pro-inflammatory cytokines (TNF-α, IL-6), hormones (insulin, leptin) and PUFAs (n-6, n-3) in blood and breastmilk in early postpartum between women with normal BMI (Group 1, n = 18; 18.

Health beliefs associated with poor disease self-management in smokers with asthma and/or COPD: a pilot study.

: Compared to nonsmokers, smokers with chronic disease are less likely to adhere to self-management recommendations for the management of their chronic conditions. Although the literature notes poor adherence trends in smokers, actual influences of adherence in these patients require further study. This study examines the health beliefs that influence self-management behaviors in smokers with chronic lung disease.

Farmers' Market Utilization among Supplemental Nutrition Assistance Program Recipients in New Orleans, Louisiana: Preliminary Findings.

Objective: Farmers' markets are increasingly being promoted as a means to provide fresh produce to poor and underserved communities. However, farmers' market (FM) use remains low among low-income patrons. The purpose of our study was to examine FM awareness and use, grocery shopping behaviors, and internet use among Supplemental Nutrition Assistance Program (SNAP) recipients.

Integrative Family Medicine - Are Louisiana Patients Ready?

Purpose: The purpose of this preliminary study was to assess patient readiness to use a family medicine clinic that integrates complementary and alternative medicine (CAM) with conventional medicine.

Methods: A survey using the theory of planned behavior as a framework addressed patient attitudes and use of CAM. Descriptive statistics provided demographic characteristics.

Applying the Social Ecological Model to Creating Asthma-Friendly Schools in Louisiana.

Background: In 2010, the Louisiana Asthma Management and Prevention Program (LAMP) implemented the Asthma-Friendly Schools Initiative in high-risk Louisiana populations. The social ecological model (SEM) was used as a framework for an asthma program implemented in 70 state K-12 public schools over 2 years.

Methods: Activities included a needs assessment, identification of students with asthma, individualized asthma action plans (AAP), staff trainings, environmental quality improvement, and school system policy changes to address the asthma burden.

Use of asthma control indicators in measuring inhaled corticosteroid effectiveness in asthmatic smokers: a systematic review.

Objective: The objective of this review is to explore how current research measures the effectiveness of inhaled corticosteroids (ICS) in smokers with asthma.

Data Sources: PubMed, CINAHL and PsycINFO databases were searched for combinations of terms relating to asthma, tobacco use and ICS effectiveness.

Study Selections: The search was limited to articles published between 2004 and 2015, in English language.

Pre-school obesity is inversely associated with vegetable intake, grocery stores and outdoor play.

The study determined the association between body mass index (BMI) z score and fruit and vegetable intake, frequency and ratio of fast food outlets and grocery stores in concentric areas around the child's residence, outdoor play and total crime index. Data from 78 Louisiana pre-school children were analyzed using Pearson's correlation and multiple regression analysis. Parental-reported fruit intake was linearly associated with increased number of grocery store counts in concentric areas around the child's residence (P = 0.

Microfluidic trap-and-release system for lab-on-a-chip-based studies on giant vesicles.

We present a microfluidic array that allows lab-on-a-chip-based studies on hundreds of giant vesicles through immobilization, engineering and release of the vesicles. Real-time observations of the vesicular response are reported. This trap-and-release system is also used to efficiently narrow the size distribution of the vesicle population.

A comparative study of the topology of the experimental electron density within 2 and 4e- donor alkyne complexes.

A comparison of the topology of the experimental electron density, as revealed by high resolution X-ray diffraction, is provided for two prototypal transition metal alkyne complexes where the alkyne formally behaves as a 2 or 4e(-) donor. A higher value of the electron density ρ(r)(bcp) at the M(T)···C bond critical point (bcp), a lower value of ρ(r)(bcp) at the coordinated C≡C bcp, outwardly bent MC bond paths and a close to zero ellipticity for the C[triple bond, length as m-dash]C bond constitute the topological signature of a 4e(-) donor alkyne ligand.

Applying the Social Ecological Model to evaluate a demonstration colorectal cancer screening program in Louisiana.

Objective: Lack of health insurance is correlated with noncompliance in colorectal cancer screening. Louisiana ranks 48th among all states in residents with health insurance. This paper describes initial results of Louisiana's first statewide colorectal cancer screening program.

Polymer vesicles as microreactors for bioinspired calcium carbonate precipitation.

Giant polymer vesicles made by electroformation have been shown to encapsulate salts up to concentrations of about 10 mM. The impermeability of these "polymersomes" to calcium ions is demonstrated by the use of fluorescent probes dedicated to calcium analysis. Permeability to calcium ions can be triggered by the addition of calcimycin, an ionophore molecule that is able to transport cations selectively through the membrane.

Matching ATP supply and demand in mammalian heart: in vivo, in vitro, and in silico perspectives.

Although the heart rapidly adapts cardiac output to match the body's circulatory demands, the regulatory mechanisms ensuring that sufficient ATP is available to perform the required cardiac work are not completely understood. Two mechanisms have been suggested to serve as key regulators: (1) ADP and Pi concentrations--ATP utilization/hydrolysis in the cytosol increases ADP and Pi fluxes to mitochondria and hence the amount of available substrates for ATP production increases; and (2) Ca2+ concentration--ATP utilization/hydrolysis is coupled to changes in free cytosolic calcium and mitochondrial calcium, the latter controlling Ca2+-dependent activation of mitochondrial enzymes taking part in ATP production. Here we discuss the evolving perspectives of each of the putative regulatory mechanisms and the precise molecular targets (dehydrogenase enzymes, ATP synthase) based on existing experimental and theoretical evidence.

Cholinergic receptor signaling modulates spontaneous firing of sinoatrial nodal cells via integrated effects on PKA-dependent Ca(2+) cycling and I(KACh).

Prior studies indicate that cholinergic receptor (ChR) activation is linked to beating rate reduction (BRR) in sinoatrial nodal cells (SANC) via 1) a G(i)-coupled reduction in adenylyl cyclase (AC) activity, leading to a reduction of cAMP or protein kinase A (PKA) modulation of hyperpolarization-activated current (I(f)) or L-type Ca(2+) currents (I(Ca,L)), respectively; and 2) direct G(i)-coupled activation of ACh-activated potassium current (I(KACh)). More recent studies, however, have indicated that Ca(2+) cycling by the sarcoplasmic reticulum within SANC (referred to as a Ca(2+) clock) generates rhythmic, spontaneous local Ca(2+) releases (LCR) that are AC-PKA dependent. LCRs activate Na(+)-Ca(2+) exchange (NCX) current, which ignites the surface membrane ion channels to effect an AP.

Role of glycogen synthase kinase-3beta in cardioprotection.

Limitation of infarct size by ischemic/pharmacological pre- and postconditioning involves activation of a complex set of cell-signaling pathways. Multiple lines of evidence implicate the mitochondrial permeability transition pore (mPTP) as a key end effector of ischemic/pharmacological pre- and postconditioning. Increasing the ROS threshold for mPTP induction enhances the resistance of cardiomyocytes to oxidant stress and results in infarct size reduction.

Regulation and pharmacology of the mitochondrial permeability transition pore.

The 'mitochondrial permeability transition', characterized by a sudden induced change of the inner mitochondrial membrane permeability for water as well as for small substances ( View Article and Find Full Text PDF

Participatory development and implementation of a community research workshop: experiences from a community-based participatory research partnership.

Background: Although community-based participatory research (CBPR) principles stress the importance of "equitable partnerships" and an "empowering and power-sharing process that attends to social inequalities," descriptions of actual projects often focus on the challenges confronted in academic-community partnerships. These challenges occur in the context of economic and power inequities and the frequently limited diversity of researchers. Less often does this discourse attend to the link between the principles of CBPR and their empowering potential for community members who internalize and use these principles to hold outside partners accountable to these ideals.

A nutrition and physical activity intervention promotes weight loss and enhances diet attitudes in low-income mothers of young children.

The purpose of this study was to evaluate a nutrition and physical activity program for reducing body weight and improving nutrition attitudes in mothers of young children. A convenience sample of 114 intervention mothers and 33 comparison mothers was recruited from public health clinics and community centers. Eligibility criteria included Hispanic, African American, or white ethnicity; body mass index of at least 25 kg/m(2); low income (< 200% of the federal poverty index); and youngest child aged 1 to 4 years.

Personalizing nutrigenomics research through community based participatory research and omics technologies.

Personal and public health information are often obtained from studies of large population groups. Risk factors for nutrients, toxins, genetic variation, and more recently, nutrient-gene interactions are statistical estimates of the percentage reduction in disease in the population if the risk were to be avoided or the gene variant were not present. Because individuals differ in genetic makeup, lifestyle, and dietary patterns than those individuals in the study population, these risk factors are valuable guidelines, but may not apply to individuals.