To send content items to your account,
please confirm that you agree to abide by our usage policies.
If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account.
Find out more about sending content to .
To send content items to your Kindle, first ensure firstname.lastname@example.org
is added to your Approved Personal Document E-mail List under your Personal Document Settings
on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part
of your Kindle email address below.
Find out more about sending to your Kindle.
Note you can select to send to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi.
‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
The initial classic Fontan utilising a direct right atrial appendage to pulmonary artery anastomosis led to numerous complications. Adults with such complications may benefit from conversion to a total cavo-pulmonary connection, the current standard palliation for children with univentricular hearts.
A single institution, retrospective chart review was conducted for all Fontan conversion procedures performed from July, 1999 through January, 2017. Variables analysed included age, sex, reason for Fontan conversion, age at Fontan conversion, and early mortality or heart transplant within 1 year after Fontan conversion.
A total of 41 Fontan conversion patients were identified. Average age at Fontan conversion was 24.5 ± 9.2 years. Dominant left ventricular physiology was present in 37/41 (90.2%) patients. Right-sided heart failure occurred in 39/41 (95.1%) patients and right atrial dilation was present in 33/41 (80.5%) patients. The most common causes for Fontan conversion included atrial arrhythmia in 37/41 (90.2%), NYHA class II HF or greater in 31/41 (75.6%), ventricular dysfunction in 23/41 (56.1%), and cirrhosis or fibrosis in 7/41 (17.1%) patients. Median post-surgical follow-up was 6.2 ± 4.9 years. Survival rates at 30 days, 1 year, and greater than 1-year post-Fontan conversion were 95.1, 92.7, and 87.8%, respectively. Two patients underwent heart transplant: the first within 1 year of Fontan conversion for heart failure and the second at 5.3 years for liver failure.
Fontan conversion should be considered early when atrial arrhythmias become common rather than waiting for severe heart failure to ensue, and Fontan conversion can be accomplished with an acceptable risk profile.
Background: Hereditary transthyretin-mediated (hATTR) amyloidosis is a multi-systemic, heterogenous, life-threatening disease. Patisiran resulted in significant improvement in neuropathy and QoL at 18-months compared to placebo, and was generally well-tolerated in the Phase 3 APOLLO study. Methods: Multi-center, OLE study to evaluate the efficacy and safety of long-term patisiran dosing for ≤ 5 years in hATTR amyloidosis patients with polyneuropathy who have completed the APOLLO study (NCT02510261). Endpoints include safety, tolerability and long-term efficacy of patisiran. Measures of clinical benefit are the same endpoints used in APOLLO including changes in mNIS+7 composite neuropathy impairment score and QoL (Norfolk QoL-DN) Results: As of December 2017, 184 of 186 (99%) patients who completed APOLLO and 25 patients from the Ph 2 OLE study enrolled in the Global OLE study. Baseline data for 211(APOLLO/placebo, n=49; APOLLO/patisiran, n=137 and patisiran Ph 2 OLE, n=25) patients included: median age 61 years (26-84); 74% males; 46% V30M. Interim safety data and 12-month efficacy results will be presented. Conclusions: The global OLE study includes a diverse population of hATTR amyloidosis patients. Interim data will include the long-term safety and maintenance of effect in patients continuing on patisiran, as well as the impact of treatment with patisiran on patients previously treated with placebo.
During the summer of 2016, the Hawaii Department of Health responded to the second-largest domestic foodborne hepatitis A virus (HAV) outbreak in the post-vaccine era. The epidemiological investigation included case finding and investigation, sequencing of RNA positive clinical specimens, product trace-back and virologic testing and sequencing of HAV RNA from the product. Additionally, an online survey open to all Hawaii residents was conducted to estimate baseline commercial food consumption. We identified 292 confirmed HAV cases, of whom 11 (4%) were possible secondary cases. Seventy-four (25%) were hospitalised and there were two deaths. Among all cases, 94% reported eating at Oahu or Kauai Island branches of Restaurant Chain A, with 86% of those cases reporting raw scallop consumption. In contrast, a food consumption survey conducted during the outbreak indicated 25% of Oahu residents patronised Restaurant Chain A in the 7 weeks before the survey. Product trace-back revealed a single distributor that supplied scallops imported from the Philippines to Restaurant Chain A. Recovery, amplification and sequence comparison of HAV recovered from scallops revealed viral sequences matching those from case-patients. Removal of product from implicated restaurants and vaccination of those potentially exposed led to the cessation of the outbreak. This outbreak further highlights the need for improved imported food safety.
We present an indentation-scope that interfaces with confocal microscopy, enabling direct observation of the three-dimensional (3D) microstructural response of coatings on substrates. Using this method, we compared microns-thick polymer coatings on glass with and without silica nanoparticle filler. Bulk force data confirmed the >30% modulus difference, while microstructural data further revealed slip at the glass-coating interface. Filled coatings slipped more and about two times faster, as reflected in 3D displacement and von Mises strain fields. Overall, these data indicate that silica-doping of coatings can dramatically alter adhesion. Moreover, this method compliments existing theoretical and modeling approaches for studying indentation in layered systems.
The increasing demand for energy storage devices has propelled research for developing efficient super-capacitors (SC) with long cycle life and ultrahigh energy density. Carbon-based materials are commonly used as electrode materials for SC. Herein, we report a new approach to improve the SC performance utilizing a Porous Carbon/Cerium Oxide nanoparticle (PC-CON) hybrid as electrode material synthesized via a low temperature hydrothermal method. Through this approach, charges can be stored not only via electrochemical double layer capacitance (EDLC) from PC but also through pseudo-capacitive effect from CeO2 nanoparticles (NPs). The electrode-electrolyte interaction due to the electrochemical properties of the electrolyte provides an enhanced voltage window for the SC. Scanning Electron Microscopy (SEM), Transmission Electron Microscopy (TEM), X-ray Photoelectron Spectroscopy (XPS), and X-Ray Diffraction (XRD) measurements were used for the characterization of this PC/CeO2 hybrid material system. The testing results have shown that a maximum of 500% higher specific capacitance could be obtained using PC/CeO2 instead of using PC only.
Introduction: High-quality cardiopulmonary resuscitation (CPR) is essential for patient survival. Typically, CPR quality is only measured during the first 10 minutes of resuscitation. There is limited research examining the quality of CPR over the entire duration of resuscitation.Objective: To examine the quality of CPR over the entire duration of resuscitation and correlate the quality of CPR to patient survival. Methods: This was a retrospective observational study using data from the Toronto RescuNET Epistry-Cardiac Arrest database. We included consecutive, adult (>18) OHCA treated by EMS between January 1, 2014 and September 30, 2015. High-quality CPR was defined, in accordance with 2015 AHA Guidelines, as a chest compression rate of 100-120/min, depth of 5.0-6.0 cm and chest compression fraction (ccf) of >0.80. We further categorized high-quality resuscitation as meeting benchmarks >80% of the time, moderate-quality between 50-80% and low-quality meeting benchmarks <50% of the resuscitation. We used multivariable logistic regression to determine association between variables of interest, including CPR quality metrics, and survival to hospital discharge. Results: A total of 5,208 OHCA met our inclusion criteria with a survival rate of 8%. The median (IQR) duration of resuscitation was 23.0 min (15.0,32.7). Overall CPR quality was considered high-quality for ccf in 81% of resuscitation episodes, 41% for rate, and 7% for depth. The percentage of resuscitations meeting the quality benchmarks differed between survivors and non-survivors for both depth (15% vs 6%) and ccf (61% vs 83%) (P value <0.001). After controlling for Utstein variables maintaining a chest compression depth within recommendations for >80% showed a trend towards improved survival (OR 1.68, 95% CI 0.96, 2.92). Other variables associated with survival were public location, initial CPR by EMS providers or bystanders, witnessed cardiac arrest (EMS or bystander), and initial shockable rhythm. Increasing age and longer duration of resuscitation were associated with decreased survival. Conclusion: Overall, EMS providers were not able to maintain rate or depth within guideline recommendations for the majority of the duration of resuscitation. Maintaining chest compression depth for greater than 80% of the resuscitation showed a trend towards increased survival from OHCA.
The monocyte–lymphocyte ratio (MLR) is a useful biomarker for disease development, but little is known about the extent to which genetic and environmental factors influence MLR variation. Here, we study the genetic architecture of MLR and determine the influence of demographic and lifestyle factors on MLR in data from a Dutch non-patient twin-family population. Data were obtained in 9,501 individuals from the Netherlands Twin Register. We used regression analyses to determine the effects of age, sex, smoking, and body mass index (BMI) on MLR and its subcomponents. Data on twins, siblings and parents (N = 7,513) were analyzed by genetic structural equation modeling to establish heritability and genome wide single nucleotide polymorphism (SNP) data from a genotyped subsample (N = 5,892) and used to estimate heritability explained by SNPs. SNP and phenotype data were also analyzed in a genome-wide association study to identify the genes involved in MLR. Linkage disequilibrium (LD) score regression and expression quantitative trait loci (eQTL) analyses were performed to further explore the genetic findings. Results showed that age, sex, and age × sex interaction effects were present for MLR and its subcomponents. Variation in MLR was not related to BMI, but smoking was positively associated with MLR. Heritability was estimated at 40% for MLR, 58% for monocyte, and 58% for lymphocyte count. The Genome-wide association study (GWAS) identified a locus on ITGA4 that was associated with MLR and only marginally significantly associated with monocyte count. For monocyte count, additional genetic variants were identified on ITPR3, LPAP1, and IRF8. For lymphocyte count, GWAS provided no significant findings. Taking all measured SNPs together, their effects accounted for 13% of the heritability of MLR, while all known and identified genetic loci explained 1.3% of variation in MLR. eQTL analyses showed that these genetic variants were unlikely to be eQTLs. In conclusion, variation in MLR level in the general population is heritable and influenced by age, sex, and smoking. We identified gene variants in the ITGA4 gene associated with variation in MLR. The significant SNP-heritability indicates that more genetic variants are likely to be involved.
Autism spectrum disorder (ASD) is a highly heritable neurodevelopmental disorder, yet the search for definite genetic etiologies remains elusive. Delineating ASD endophenotypes can boost the statistical power to identify the genetic etiologies and pathophysiology of ASD. We aimed to test for endophenotypes of neuroanatomy and associated intrinsic functional connectivity (iFC) via contrasting male youth with ASD, their unaffected brothers and typically developing (TD) males.
The 94 participants (aged 9–19 years) – 20 male youth with ASD, 20 unaffected brothers and 54 TD males – received clinical assessments, and undertook structural and resting-state functional magnetic resonance imaging scans. Voxel-based morphometry was performed to obtain regional gray and white matter volumes. A seed-based approach, with seeds defined by the regions demonstrating atypical neuroanatomy shared by youth with ASD and unaffected brothers, was implemented to derive iFC. General linear models were used to compare brain structures and iFC among the three groups. Assessment of familiality was investigated by permutation tests for variance of the within-family pair difference.
We found that atypical gray matter volume in the mid-cingulate cortex was shared between male youth with ASD and their unaffected brothers as compared with TD males. Moreover, reduced iFC between the mid-cingulate cortex and the right inferior frontal gyrus, and increased iFC between the mid-cingulate cortex and bilateral middle occipital gyrus were the shared features of male ASD youth and unaffected brothers.
Atypical neuroanatomy and iFC surrounding the mid-cingulate cortex may be a potential endophenotypic marker for ASD in males.
Malaysia introduced graphic health warning labels (GHWLs) on all tobacco packages in 2009. We aimed to examine if implementing GHWLs led to stronger warning reactions (e.g., thinking about the health risks of smoking) and an increase in subsequent quitting activities; and to examine how reactions changed over time since the implementation of the GHWLs in Malaysia and Thailand where GHWL size increased from 50–55% in 2010. Data came from six waves (2005–2014) of the International Tobacco Control Southeast Asia Survey. Between 3,706 and 4,422 smokers were interviewed across these two countries at each survey wave. Measures included salience of warnings, cognitive responses (i.e., thinking about the health risks and being more likely to quit smoking), forgoing cigarettes, and avoiding warnings. The main outcome was subsequent quit attempts. Following the implementation of GHWLs in Malaysia, reactions increased, in some cases to levels similar to the larger Thai warnings, but declined over time. In Thailand, reactions increased following implementation, with no decline for several years, and no clear effect of the small increase in warning size. Reactions, mainly cognitive responses, were consistently predictive of quit attempts in Thailand, but this was only consistently so in Malaysia after the change to GHWLs. In conclusion, GHWLs are responded to more frequently, and generate more quit attempts, but warning wear-out is not consistent in these two countries, perhaps due to differences in other tobacco control efforts.
The phenomena of surface instabilities in a thin power-law fluid during spin coating are investigated. The set of Navier-Stokes equations with non-Newtonian behavior in the region of low Reynolds number serves as a mathematical description of the physical systems. Long-wave perturbation analysis is proposed to derive an evolution equation of the Ostwald de-Waele type fluid to govern the propagation of surface waves. Weakly nonlinear dynamics of film flow is studied by the multiple scales method. The amplitude of instability is determined by a Ginzburg-Landau equation. The study reveals that the degree of power-law index plays a vital role in stabilizing the film flow. The shear-thinning fluid is more unstable than the shear-thickening fluid in the stability analysis. Further, the nonlinear wave speed in the supercritical stability region decreases with increasing values of power-law index.
Optical control is a reversible and convenient technology, able to be measured in real-time, which makes it excellent for application to microfluidic, biomechanical, and electro-mechanical devices. These advantages are especially attractive for photo-responsive materials. In this study, we developed a new photo-responsive, electrostrictive material from a composite material made by mixing a dielectric polymer P(VDF-TrFE-CFE) and an organic photoconductive material TiOPc. The photo-responsibility of the material has been validated by corresponding actuators. We found that under white light illumination, deformation will increase which can be attributed to a decrease in the TiOPc impedance. We identified that the optimal TiOPc concentration for actuator applications is 10% P(VDF-TrFE-CFE)/TiOPc. Moreover, controlling the fluid flow within the capillary tube through light illumination also validated the photo-responsive actuator. Our results show that the mechanism and the photo-responsive material can be used to pursue further study on light controlling microfluidic, and related electro-mechanical devices.
Increased intra-individual variability (IIV) in reaction time (RT) across various tasks is one ubiquitous neuropsychological finding in attention deficit hyperactivity disorder (ADHD). However, neurobiological underpinnings of IIV in individuals with ADHD have not yet been fully delineated. The ex-Gaussian distribution has been proved to capture IIV in RT. The authors explored the three parameters [μ (mu), σ (sigma), τ (tau)] of an ex-Gaussian RT distribution derived from the Conners' continuous performance test (CCPT) and their correlations with the microstructural integrity of the frontostriatal–caudate tracts and the cingulum bundles.
We assessed 28 youths with ADHD (8–17 years; 25 males) and 28 age-, sex-, IQ- and handedness-matched typically developing (TD) youths using the CCPT, Wechsler Intelligence Scale for Children, 3rd edition and magnetic resonance imaging (MRI). Microstructural integrity, indexed by generalized fractional anisotropy (GFA), was measured by diffusion spectrum imaging tractrography on a 3-T MRI system.
Youths with ADHD had larger σ (s.d. of Gaussian distribution) and τ (mean of exponential distribution) and reduced GFA in four bilateral frontostriatal tracts. With increased inter-stimulus intervals of CCPT, the magnitude of greater τ in ADHD than TD increased. In ADHD youths, the cingulum bundles and frontostriatal integrity were associated with three ex-Gaussian parameters and with μ (mean of Gaussian distribution) and τ, respectively; while only frontostriatal GFA was associated with μ and τ in TD youths.
Our findings suggest the crucial role of the integrity of the cingulum bundles in accounting for IIV in ADHD. Involvement of different brain systems in mediating IIV may relate to a distinctive pathophysiological processing and/or adaptive compensatory mechanism.
The UFFO (Ultra-Fast Flash Observatory) is a GRB detector on board the Lomonosov
satellite, to be launched in 2013. The GRB trigger is provided by an X-ray detector,
called UBAT (UFFO Burst Alarm & Trigger Telescope), which detects X-rays from the GRB
and then triggers to determine the direction of the GRB and then alerts the Slewing Mirror
Telescope (SMT) to turn in the direction of the GRB and record the optical photon fluxes.
This report details the calibration of the two components: the MAPMTs and the YSO crystals
and simulations of the UBAT. The results shows that this design can observe a GRB within a
field of view of ±35° and can trigger in a time scale as short as 0.2 – 1.0 s
after the appearance of a GRB X-ray spike.
The Ultra-Fast Flash Observatory (UFFO) Pathfinder for Gamma-Ray Bursts (GRBs) consists
of two telescopes. The UFFO Burst Alert & Trigger Telescope (UBAT) handles the
detection and localization of GRBs, and the Slewing Mirror Telescope (SMT) conducts the
measurement of the UV/optical afterglow. UBAT is equipped with an X-ray detector, analog
and digital signal readout electronics that detects X-rays from GRBs and determines the
location. SMT is equipped with a stepping motor and the associated electronics to rotate
the slewing mirror targeting the GRBs identified by UBAT. First the slewing mirror points
to a GRB, then SMT obtains the optical image of the GRB using the intensified CCD and its
readout electronics. The UFFO Data Acquisition system (UDAQ) is responsible for the
overall function and operation of the observatory and the communication with the satellite
main processor. In this paper we present the design and implementation of the electronics
of UBAT and SMT as well as the architecture and implementation of UDAQ.
The Ultra-Fast Flash Observatory (UFFO), which will be launched onboard the
Lomonosov spacecraft, contains two crucial instruments: UFFO Burst
Alert & Trigger Telescope (UBAT) for detection and localization of Gamma-Ray Bursts
(GRBs) and the fast-response Slewing Mirror Telescope (SMT) designed for the observation
of the prompt optical/UV counterparts. Here we discuss the in-space calibrations of the
UBAT detector and SMT telescope. After the launch, the observations of the standard X-ray
sources such as pulsar in Crab nebula will provide data for necessary calibrations of
UBAT. Several standard stars will be used for the photometric calibration of SMT. The
celestial X-ray sources, e.g. X-ray binaries with bright optical sources
in their close angular vicinity will serve for the cross-calibration of UBAT and SMT.