A service evaluation of parent adherence with dysphagia management therapy guidelines: reports from family carers supporting children with complex needs in Greece.

Many children with complex needs exhibit eating, drinking, and/or swallowing disorders (dysphagia). These children often have associated learning needs, and require assistance from carers for daily tasks such as eating and drinking. The aim of this study was to identify which strategies to manage dysphagia were challenging for family carers, and reasons for any non-adherence.

Pharmacokinetics of glycerol phenylbutyrate in pediatric patients 2 months to 2 years of age with urea cycle disorders.

Introduction: Glycerol phenylbutyrate (GPB) is approved in the US and EU for the chronic management of patients ≥2 months of age with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone. GPB is a pre-prodrug, hydrolyzed by lipases to phenylbutyric acid (PBA) that upon absorption is beta-oxidized to the active nitrogen scavenger phenylacetic acid (PAA), which is conjugated to glutamine (PAGN) and excreted as urinary PAGN (UPAGN). Pharmacokinetics (PK) of GPB were examined to see if hydrolysis is impaired in very young patients who may lack lipase activity.

Ultrastructural changes in peripheral blood leukocytes in α-synuclein knockout mice.

Effects of α-synuclein deficiency on cellular blood components have not been extensively investigated. This study evaluated ultrastructural changes of leukocytes in α-synuclein knockout (KO) mice using electron microscopy (EM). The following ultrastructural characteristics were quantified in leukocytes: mitochondria, primary granules, specific granules (SG), Golgi apparatus (GA), inclusions, rough-endoplasmic reticulum (RER), smooth-endoplasmic reticulum (SER), and cellular projections (CP).

What research is needed to validate new urodynamic methods? ICI-RS2017.

Aims: In recent years urodynamic innovations, although well researched, have failed to follow a standardized development pathway specifically in terms of clinical trials undertaken to demonstrate efficacy. This was discussed at the International Consultation on Incontinence Research Society (ICI-RS) in Bristol, United Kingdom, 2017 with the aim of defining minimum standards for future urodynamic research.

Methods: The recent recommendations from the IDEAL (innovation, development, exploration, assessment, and long-term study) collaboration regarding surgical research were reviewed.

Do we understand voiding dysfunction in women? Current understanding and future perspectives: ICI-RS 2017.

Aims: Urinary retention in women is poorly understood, compared to the equivalent condition in men, and was the subject of a dedicated session organized at the International Consultation on Incontinence Research Society (ICI-RS) in Bristol, United Kingdom, 2017.

Methods: The current understanding of the pathophysiological correlates of idiopathic voiding dysfunction in women, the role of urodynamics, neurophysiology, and non-invasive tests in characterizing functional bladder outlet obstruction due to a non-relaxing urethral sphincter, and poorly defined diagnosis of detrusor underactivity were reviewed.

Results: Putative factors underlying the pathogenesis of urinary retention following urinary tract infections were discussed.

α-Synuclein concentration increases over time in plasma supernatant of single donor platelets.

Objectives: In platelets, α-synuclein is important in calcium-dependent granule release. Notably, cells release α-synuclein in setting of cell damage or death. Therefore, we investigated α-synuclein levels in plasma of single donor platelet (SDP) units during storage.

Mycobacterium tuberculosis Lipoprotein and Lipoglycan Binding to Toll-Like Receptor 2 Correlates with Agonist Activity and Functional Outcomes.

causes persistent infection due to its ability to evade host immune responses. induces Toll-like receptor 2 (TLR2) signaling, which influences immune responses to TLR2 agonists expressed by include lipoproteins (e.g.

Simulation for emergency nurses (SIREN): A quasi-experimental study.

Background: Within nursing education, simulation has been recognised as an effective learning strategy. Embedding simulation within clinical units has the potential to enhance patient safety and improve clinical outcomes. However it is important to evaluate the effectiveness of this educational technique to support the actual value and effectiveness.

Detailed analysis of patient-reported lower urinary tract symptoms and effect on quality of life after robotic radical prostatectomy.

Objective: To prospectively evaluate short- to medium-term patient-reported lower urinary tract symptoms (LUTS) and their effect on health-related quality of life (HRQoL) using validated questionnaires in a large cohort of patients following robotic-assisted radical prostatectomy (RARP) for prostate cancer.

Materials And Methods: HRQoL and LUTS outcomes were prospectively assessed in 357 consecutive men undergoing RARP at a single center from 2012 to 2015 using the functional assessment of cancer therapy-prostate (FACT-P) and the international consultation on incontinence modular questionnaire-male LUTS (ICIQ-MLUTS). Questionnaires were administered at baseline, 6, 12, and 18 months.

Distinct amino acid residues confer one of three UDP-sugar substrate specificities in Acinetobacter baumannii PglC phosphoglycosyltransferases.

Acinetobacter baumannii is an opportunistic human pathogen with the highest reported rates of multidrug resistance among Gram-negative pathogens. The capsular polysaccharide of A. baumannii is considered one of its most significant virulence factors providing resistance against complemented-mediated killing.

Pegvaliase for the treatment of phenylketonuria: Results of a long-term phase 3 clinical trial program (PRISM).

Background: Phenylketonuria (PKU) is caused by phenylalanine hydroxylase (PAH) deficiency that results in phenylalanine (Phe) accumulation. Pegvaliase, PEGylated recombinant Anabaena variabilis phenylalanine ammonia lyase (PAL), converts Phe to trans-cinnamic acid and ammonia, and is a potential enzyme substitution therapy to lower blood Phe in adults with PKU.

Methods: Two Phase 3 studies, PRISM-1 and PRISM-2, evaluated the efficacy and safety of pegvaliase treatment using an induction, titration, and maintenance dosing regimen in adults with PKU.

Pegvaliase for the treatment of phenylketonuria: A pivotal, double-blind randomized discontinuation Phase 3 clinical trial.

Introduction: Pegvaliase is a recombinant Anabaena variabilis phenylalanine ammonia lyase (PAL) enzyme under investigation for treatment of adult phenylketonuria (PKU). This manuscript describes results of a randomized discontinuation trial (RDT) designed to evaluate the effects of pegvaliase treatment on blood phenylalanine (Phe) and neuropsychiatric outcomes in adults with PKU.

Methods: PRISM-2 is a 4-part, Phase 3 study that enrolled adults with PKU receiving pegvaliase treatment (initiated in a prior Phase 2 or Phase 3 study).

Buddhism, Christianity, and psychotherapy: A three-way conversation in the mid-twentieth century.

This article explores the scope of 'religion-psy dialogue' in the mid-twentieth century, via a case study from Japan: Kosawa Heisaku, a Buddhist psychoanalyst based in Tokyo. By putting this case study in brief comparative perspective, with the conversation that took place in 1965 between Paul Tillich and Carl Rogers, the article discusses both the promise and the pitfalls of the modern and contemporary world of 'religion-psy dialogue', alongside the means by which specialists in a variety of fields might investigate and hold it to account.

Tetrahydrobiopterin treatment reduces brain L-Phe but only partially improves serotonin in hyperphenylalaninemic ENU1/2 mice.

Hyperphenylalaninemia (HPA) caused by hepatic phenylalanine hydroxylase (PAH) deficiency has severe consequences on brain monoamine neurotransmitter metabolism. We have studied monoamine neurotransmitter status and the effect of tetrahydrobiopterin (BH) treatment in Pah (ENU1/2) mice, a model of partial PAH deficiency. These mice exhibit elevated blood L-phenylalanine (L-Phe) concentrations similar to that of mild hyperphenylalaninemia (HPA), but brain levels of L-Phe are still ~5-fold elevated compared to wild-type.

Male synthetic sling versus artificial urinary sphincter trial for men with urodynamic stress incontinence after prostate surgery (MASTER): study protocol for a randomised controlled trial.

Background: Stress urinary incontinence (SUI) is a frequent adverse effect for men undergoing prostate surgery. A large proportion (around 8% after radical prostatectomy and 2% after transurethral resection of prostate (TURP)) are left with severe disabling incontinence which adversely effects their quality of life and many are reliant on containment measures such as pads (27% and 6% respectively). Surgery is currently the only option for active management of the problem.

Increasing the knowledge, identification and treatment of osteoporosis through education and shared decision-making with residents living in a retirement village community.

Objective: This pilot study explored whether individual goal setting in a retirement village setting could improve strategies to strengthen bones in an ageing population and help prevent osteoporosis.

Methods: A two-phased osteoporosis prevention program was developed, piloted and evaluated involving a group education session followed by the development of individualised Bone Plans based upon personal understanding of individual fracture risk and lifestyle factors.

Results: A significant improvement in knowledge and understanding of factors to prevent and manage osteoporosis was achieved, and changes in lifestyle behaviours were sustained at six months.

Blood phenylalanine reduction corrects CNS dopamine and serotonin deficiencies and partially improves behavioral performance in adult phenylketonuric mice.

Central nervous system (CNS) deficiencies of the monoamine neurotransmitters dopamine and serotonin have been implicated in the pathophysiology of neuropsychiatric dysfunction in human phenylketonuria (PKU). In this study, we confirmed the occurrence of brain dopamine and serotonin deficiencies in association with severe behavioral alterations and cognitive impairments in hyperphenylalaninemic C57BL/6-Pah mice, a model of human PKU. Phenylalanine-reducing treatments, including either dietary phenylalanine restriction or liver-directed gene therapy, initiated during adulthood were associated with increased brain monoamine content along with improvements in nesting behavior but without a change in the severe cognitive deficits exhibited by these mice.

How can we maximize the diagnostic utility of uroflow?: ICI-RS 2017.

Aims: To gauge the current level of diagnostic utility of uroflowmetry and to suggest areas needing research to improve this.

Methods: A summary of the debate held at the 2017 meeting of the International Consultation on Incontinence Research Society, with subsequent analysis by the authors.

Results: Limited diagnostic sensitivity and specificity exist for maximum flow rates, multiple uroflow measurements, and flow-volume nomograms.

Uncovering the mechanisms of Acinetobacter baumannii virulence.

Acinetobacter baumannii is a nosocomial pathogen that causes ventilator-associated as well as bloodstream infections in critically ill patients, and the spread of multidrug-resistant Acinetobacter strains is cause for concern. Much of the success of A. baumannii can be directly attributed to its plastic genome, which rapidly mutates when faced with adversity and stress.

Using non-nutritive sucking to support feeding development for premature infants: A commentary on approaches and current practice.

Non-nutritive sucking is often used with premature infants by either using a pacifier or an expressed breast nipple to support the introduction and development of early oral feeding. The pattern of non-nutritive sucking is distinct in that it involves two sucks per second in contrast to nutritive sucking which is one suck per second. Although some literature has identified that non-nutritive sucking has some benefit for the premature infant's feeding development, it is not entirely clear why such an approach is helpful as neurologically, activation of non-nutritive and nutritive skills are different.

Optimizing patient education of oncology medications: A descriptive survey of pharmacist-provided patient education in Canada.

Background: The incidence of cancer is increasing in Canada due to an aging and growing population. This frequently necessitates chemotherapy, which is a high-risk treatment, often given as a part of a complex regimen with serious side effects. A review of the evidence of pharmacy-provided patient education initiatives targeted to oncology patients revealed that minimal is known about this service.

Proteomics and Network Analyses Reveal Inhibition of Akt-mTOR Signaling in CD4 T Cells by Mycobacterium tuberculosis Mannose-Capped Lipoarabinomannan.

Mycobacterium tuberculosis (Mtb) cell wall glycolipid mannose-capped lipoarabinomannan (ManLAM) inhibits CD4 T-cell activation by inhibiting proximal T-cell receptor (TCR) signaling when activated by anti-CD3. To understand the impact of ManLAM on CD4 T-cell function when both the TCR-CD3 complex and major costimulator CD28 are engaged, we performed label-free quantitative MS and network analysis. Mixed-effect model analysis of peptide intensity identified 149 unique peptides representing 131 proteins that were differentially regulated by ManLAM in anti-CD3- and anti-CD28-activated CD4 T cells.