Image texture features predict renal function decline in patients with autosomal dominant polycystic kidney disease.

Magnetic resonance imaging (MRI) examinations provide high-resolution information about the anatomic structure of the kidneys and are used to measure total kidney volume (TKV) in patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD). Height-adjusted TKV (HtTKV) has become the gold-standard imaging biomarker for ADPKD progression at early stages of the disease when estimated glomerular filtration rate (eGFR) is still normal. However, HtTKV does not take advantage of the wealth of information provided by MRI.

Polycystic Kidney Disease without an Apparent Family History.

The absence of a positive family history (PFH) in 10%-25% of patients poses a diagnostic challenge for autosomal dominant polycystic kidney disease (ADPKD). In the Toronto Genetic Epidemiology Study of Polycystic Kidney Disease, 210 affected probands underwent renal function testing, abdominal imaging, and comprehensive and mutation screening. From this cohort, we reviewed all patients with and without an apparent family history, examined their parental medical records, and performed renal imaging in all available parents of unknown disease status.

Experimentally Manipulated Self-Affirmation Promotes Reduced Alcohol Consumption in Response to Narrative Information.

Background: Health-risk information is increasingly being conveyed through accounts of personal experiences or narrative information. However, whether self-affirmation can enhance the ability of such messages to promote behavior change has yet to be established.

Purpose: This study aims to test whether self-affirmation (a) promotes behavior change following exposure to narrative information about the risks of excessive alcohol consumption and (b) boosts message acceptance by increasing narrative engagement.

Effect of Statin Therapy on the Progression of Autosomal Dominant Polycystic Kidney Disease. A Secondary Analysis of the HALT PKD Trials.

Background: Autosomal dominant polycystic kidney disease (ADPKD) commonly results in end-stage renal disease (ESRD), yet a long-term treatment that is well tolerated is still lacking. In a small randomized trial in children and adolescents pravastatin administration for 3 years was associated with reduced renal cyst growth, but no large trial has tested the effect of statins in adults.

Methods: We performed a post-hoc analysis of the HALT PKD trials to compare outcomes of participants who never used statins with those who used statin for at least 3 years.

Diurnal patterns and relationships between physiological and self-reported stress in patients with epilepsy and psychogenic non-epileptic seizures.

Purpose: Patients with epilepsy and those with psychogenic non-epileptic seizures (PNES) experience high levels of stress and stress is one of the most frequently self-identified seizure precipitants. Although stress is a multifaceted phenomenon, few studies have systematically examined its different components in patients with seizures. The aim of this study was therefore to describe diurnal patterns of psychological and physiological measures of stress in patients with epilepsy and patients with PNES, and explore their relationships to each other in order to improve our understanding of the mechanisms underlying stress and seizure occurrence in these patients.

B-type natriuretic peptide overexpression ameliorates hepatorenal fibrocystic disease in a rat model of polycystic kidney disease.

Polycystic kidney disease (PKD) involves progressive hepatorenal cyst expansion and fibrosis, frequently leading to end-stage renal disease. Increased vasopressin and cAMP signaling, dysregulated calcium homeostasis, and hypertension play major roles in PKD progression. The guanylyl cyclase A agonist, B-type natriuretic peptide (BNP), stimulates cGMP and shows anti-fibrotic, anti-hypertensive, and vasopressin-suppressive effects, potentially counteracting PKD pathogenesis.

PKD2-Related Autosomal Dominant Polycystic Kidney Disease: Prevalence, Clinical Presentation, Mutation Spectrum, and Prognosis.

Background: PKD2-related autosomal dominant polycystic kidney disease (ADPKD) is widely acknowledged to be of milder severity than PKD1-related disease, but population-based studies depicting the exact burden of the disease are lacking. We aimed to revisit PKD2 prevalence, clinical presentation, mutation spectrum, and prognosis through the Genkyst cohort.

Study Design: Case series, January 2010 to March 2016.

Characterizing Sirtuin 3 Deacetylase Affinity for Aldehyde Dehydrogenase 2.

Mitochondrial aldehyde dehydrogenase (ALDH2) plays a central role in the detoxification of reactive aldehydes generated through endogenous and exogenous sources. The biochemical regulation of enzyme activity through post-translational modification provides an intricate response system regulating mitochondrial detoxification pathways. ALDH2 is a known target of lysine acetylation, which arises as a consequence of mitochondrial bioenergetic flux and sirtuin deacetylase activity.

microRNA-17 family promotes polycystic kidney disease progression through modulation of mitochondrial metabolism.

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent genetic cause of renal failure. Here we identify miR-17 as a target for the treatment of ADPKD. We report that miR-17 is induced in kidney cysts of mouse and human ADPKD.

Distinguishing between Hepatic Inflammation and Fibrosis with MR Elastography.

Purpose To investigate the utility of magnetic resonance (MR) elastography-derived mechanical properties in the discrimination of hepatic inflammation and fibrosis in the early stages of chronic liver diseases. Materials and Methods All studies were approved by the institutional animal care and use committee. A total of 187 animals were studied, including 182 mice and five pigs.

Parallel microarray profiling identifies ErbB4 as a determinant of cyst growth in ADPKD and a prognostic biomarker for disease progression.

Autosomal dominant polycystic kidney disease (ADPKD) is the fourth most common cause of end-stage renal disease. The disease course can be highly variable and treatment options are limited. To identify new therapeutic targets and prognostic biomarkers of disease, we conducted parallel discovery microarray profiling in normal and diseased human cystic kidney cells.

Digenic mutations of human paralogs in Dent's disease type 2 associated with Chiari I malformation.

and its paralog encode phosphatidylinositol 5-phosphatases that localize to the primary cilium and have roles in ciliogenesis. Mutations in cause the X-linked Dent disease type 2 (DD2; OMIM# 300555), characterized by low-molecular weight proteinuria, hypercalciuria, and the variable presence of cataracts, glaucoma and intellectual disability without structural brain anomalies. Disease-causing mutations in have not been described in humans.

Autophagy activators suppress cystogenesis in an autosomal dominant polycystic kidney disease model.

Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in either PKD1 or PKD2. It is one of the most common heritable human diseases with eventual development of renal failure; however, effective treatment is lacking. While inhibition of mechanistic target of rapamycin (mTOR) effectively slows cyst expansions in animal models, results from clinical studies are controversial, prompting further mechanistic studies of mTOR-based therapy.

Quantitative Analysis and Discovery of Lysine and Arginine Modifications.

Post-translational modifications (PTMs) affect protein function, localization, and stability, yet very little is known about the ratios of these modifications. Here, we describe a novel method to quantitate and assess the relative stoichiometry of Lys and Arg modifications (QuARKMod) in complex biological settings. We demonstrate the versatility of this platform in monitoring recombinant protein modification of peptide substrates, PTMs of individual histones, and the relative abundance of these PTMs as a function of subcellular location.

Increasing engagement with, and effectiveness of, an online CBT-based stress management intervention for employees through the use of an online facilitated bulletin board: study protocol for a pilot randomised controlled trial.

Background: The evidence for the benefits of online cognitive behaviour therapy (CBT)-based programmes delivered in a clinical context is clear, but this evidence does not translate to online CBT-based stress management programmes delivered within a workplace context. One of the challenges to the delivery of online interventions is programme engagement; this challenge is even more acute for interventions delivered in real-world settings such as the workplace. The purpose of this pilot study is to explore the effect of an online facilitated discussion group on engagement, and to estimate the potential effectiveness of an online CBT-based stress management programme.

Social cohesion: The missing factor required for a successful hand hygiene program.

Background: There are limited explorations into hospital staff reactions to automated hand hygiene surveillance or hand hygiene interventions.

Methods: An automated surveillance system with daily feedback and a behavioral intervention component was trialed in 2 wards in an Australian tertiary teaching hospital. After 9 months, 12 clinicians from each ward were interviewed prior to the completion of the trial to explore satisfaction with the system and behavioral component of nudging each other with a reminder to comply.

Standardisation of a European measurement method for the determination of anions and cations in PM: results of field trial campaign and determination of measurement uncertainty.

European Committee for Standardisation (CEN) Technical Committee 264 'Air Quality' has recently produced a standard method for the measurements of anions and cations in PM within its Working Group 34 in response to the requirements of European Directive 2008/50/EC. It is expected that this method will be used in future by all Member States making measurements of the ionic content of PM. This paper details the results of a field measurement campaign and the statistical analysis performed to validate this method, assess its uncertainty and define its working range to provide clarity and confidence in the underpinning science for future users of the method.

Risk factors and mortality associated with undertriage at a level I safety-net trauma center: a retrospective study.

Purpose: The primary objective of this study was to determine the risk factors associated with undertriage and the risk factors for mortality among the undertriaged patients at a level I safety-net trauma center.

Methods: A retrospective analysis was performed of all trauma patients who presented to a level I safety-net trauma center with an injury severity score >15 over a 2-year period (2013-2014). Univariate and multivariate regression analyses were used to determine the risk factors predictive of undertriage in major trauma patients (injury severity score >15) and of mortality in undertriaged patients.

Transcriptome analysis reveals manifold mechanisms of cyst development in ADPKD.

Background: Autosomal dominant polycystic kidney disease (ADPKD) causes progressive loss of renal function in adults as a consequence of the accumulation of cysts. ADPKD is the most common genetic cause of end-stage renal disease. Mutations in polycystin-1 occur in 87% of cases of ADPKD and mutations in polycystin-2 are found in 12% of ADPKD patients.

The structure of junctions between carbon nanotubes and graphene shells.

Junctions between carbon nanotubes and flat or curved graphene structures are fascinating for a number of reasons. It has been suggested that such junctions could be used in nanoelectronic devices, or as the basis of three-dimensional carbon materials, with many potential applications. However, there have been few detailed experimental analyses of nanotube-graphene connections.

DRH1, a p68-related RNA helicase gene, is required for chromosome breakage in Tetrahymena.

The p68 DEAD box helicases comprise a widely conserved protein family involved in a large range of biological processes including transcription, splicing and translation. The genome of the ciliate Tetrahymena thermophile encodes two p68-like helicases, Drh1p and Lia2p. We show that DRH1 is essential for growth and completion of development.

Processing of Alcohol-Related Health Threat in At-Risk Drinkers: An Online Study of Gender-Related Self-Affirmation Effects.

Aims: Defensiveness in response to threatening health information related to excessive alcohol consumption prevents appropriate behaviour change. Alternatively, self-affirmation may improve cognitive-affective processing of threatening information, thus contributing to successful self-regulation.

Methods: Effects of an online self-affirmation procedure were examined in at-risk university student drinkers.

MPS1 kinase as a potential therapeutic target in medulloblastoma.

Medulloblastoma is the most common type of malignant brain tumor that affects children. Although recent advances in chemotherapy and radiation have improved outcomes, high-risk patients perform poorly with significant morbidity. Gene expression profiling has revealed that monopolar spindle 1 (MPS1) (TTK1) is highly expressed in medulloblastoma patient samples compared to that noted in normal cerebellum.

Glomerular Pathology in Dent Disease and Its Association with Kidney Function.

Background And Objectives: Dent disease is a rare X-linked disorder characterized by low molecular weight proteinuria and often considered a renal tubular disease. However, glomerulosclerosis was recently reported in several patients. Thus, Dent disease renal histopathologic features were characterized and assessed, and their association with kidney function was assessed.