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Mitochondrial trifunctional protein deficiency is a long-chain fatty acid disorder that may include manifestations of severe cardiomyopathy and arrhythmias. The pathophysiology for the severe presentation is unclear but is an indicator for worse outcomes. Triheptanoin, a synthetic medium chain triglyceride, has been reported to reverse cardiomyopathy in some individuals, but there is limited literature in severe cases. We describe a neonatal onset of severe disease whose clinical course was not improved despite mechanical support and triheptanoin.
Publicly available health information is increasingly important for patients and their families. While the average US citizen reads at an 8th-grade level, electronic educational materials for patients and families are often advanced. We assessed the quality and readability of publicly available resources regarding hypoplastic left heart syndrome (HLHS).
Methods:
We queried four search engines for “hypoplastic left heart syndrome”, “HLHS”, and “hypoplastic left ventricle”. The top 30 websites from searches on Google, Yahoo!, Bing, and Dogpile were combined into a single list. Duplicates, commercial websites, physician-oriented resources, disability websites, and broken links were removed. Websites were graded for accountability, content, interactivity, and structure using a two-reviewer system. Nonparametric analysis of variance was performed.
Results:
Fifty-two websites were analysed. Inter-rater agreement was high (Kappa = 0.874). Website types included 35 hospital/healthcare organisation (67.3%), 12 open access (23.1%), 4 governmental agency (7.7%), and 1 professional medical society (1.9%). Median total score was 19 of 39 (interquartile range = 15.8–25.3): accountability 5.5 of 17 (interquartile range = 2.0–9.3), content 8 of 12 (interquartile range = 6.4–10.0), interactivity 2 of 6 (interquartile range = 2.0–3.0), and structure 3 of 4 (interquartile range = 2.8–4.0). Accountability was low with 32.7% (n = 17) of sites disclosing authorship and 26.9% (n = 14) citing sources. Forty-two percent (n = 22) of websites were available in Spanish. Total score varied by website type (p = 0.03), with open access sites scoring highest (median = 26.5; interquartile range = 20.5–28.6) and hospital/healthcare organisation websites scoring lowest (median = 17.5; interquartile range = 13.5–21.5). Score differences were driven by differences in accountability (p = 0.001) – content scores were similar between groups (p = 0.25). Overall readability was low, with median Flesch–Kincaid Grade Level of 11th grade (interquartile range = 10th–12th grade).
Conclusions:
Our evaluation of popular websites about HLHS identifies multiple opportunities for improvement, including increasing accountability by disclosing authorship and citing sources, enhancing readability by providing material that is understandable to readers with the full spectrum of educational background, and providing information in languages besides English, all of which would enhance health equity.
The dynamics of interfaces in slow diffusion equations with strong absorption are studied. Asymptotic methods are used to give descriptions of the behaviour local to a comprehensive range of possible singular events that can occur in any evolution. These events are: when an interface changes its direction of propagation (reversing and anti-reversing), when an interface detaches from an absorbing obstacle (detaching), when two interfaces are formed by film rupture (touchdown) and when the solution undergoes extinction. Our account of extinction and self-similar reversing and anti-reversing is built upon previous work; results on non-self-similar reversing and anti-reversing and on the various types of detachment and touchdown are developed from scratch. In all cases, verification of the asymptotic results against numerical solutions to the full PDE is provided. Self-similar solutions, both of the full equation and of its asymptotic limits, play a central role in the analysis.
This paper documents the patterns and correlates of retirement in China using a nationally representative survey, the China Health and Retirement Longitudinal Study. After documenting stark differences in retirement ages between urban and rural residents, the paper shows that China's urban residents retire earlier than workers in many Organization for Economic Co-operation and Development countries and that rural residents continue to work until advanced ages. Differences in access to generous pensions and economic resources explain much of the urban–rural difference in retirement rates. The paper suggests that reducing disincentives created by China's Urban Employee Pension system, improving health status, providing childcare and elder care support may all facilitate longer working lives. Given spouse preferences for joint retirement, creating incentives for women to retire later may facilitate longer working lives for both men and women.
The genetics of the root system is still not dissected for wheat and lack of knowledge prohibits the use of marker-assisted selection in breeding. To understand the genetic mechanism of root development, Synthetic W7984 × Opata M85 doubled-haploid (SynOpDH) mapping population was evaluated for root and shoot characteristics in PVC tubes until maturity. Two major quantitative trait loci (QTLs) for total root biomass were detected on homoeologous chromosomes 2A and 2D with logarithm of the odds scores between 6.25–10.9 and 11.8–20.86, and total phenotypic effects between 12.7–17.7 and 26.6–40.04% in 2013 and 2014, respectively. There was a strong correlation between days to anthesis and root and shoot biomass accumulation (0.50–0.81). The QTL for biomass traits on chromosome 2D co-locates with QTL for days to anthesis. The effect of extended vegetative growth, caused by photoperiod sensitivity (Ppd) genes, on biomass accumulation was always hypothesized, this is the first study to genetically support this theory.
The disease- and mortality-related difference between biological age based on DNA methylation and chronological age (Δage) has been found to have approximately 40% heritability by assuming that the familial correlation is only explained by additive genetic factors. We calculated two different Δage measures for 132 middle-aged female twin pairs (66 monozygotic and 66 dizygotic twin pairs) and their 215 sisters using DNA methylation data measured by the Infinium HumanMethylation450 BeadChip arrays. For each Δage measure, and their combined measure, we estimated the familial correlation for MZ, DZ and sibling pairs using the multivariate normal model for pedigree analysis. We also pooled our estimates with those from a former study to estimate weighted average correlations. For both Δage measures, there was familial correlation that varied across different types of relatives. No evidence of a difference was found between the MZ and DZ pair correlations, or between the DZ and sibling pair correlations. The only difference was between the MZ and sibling pair correlations (p < .01), and there was marginal evidence that the MZ pair correlation was greater than twice the sibling pair correlation (p < .08). For weighted average correlation, there was evidence that the MZ pair correlation was greater than the DZ pair correlation (p < .03), and marginally greater than twice the sibling pair correlation (p < .08). The varied familial correlation of Δage is not explained by additive genetic factors alone, implying the existence of shared non-genetic factors explaining variation in Δage for middle-aged women.
A series of laser pump, x-ray probe experiments show that above band gap photoexcitation can generate a large out-of-plane strain in multiferroic BiFeO3 thin films. The strain decays in a time scale that is the same as the photo-induced carriers measured in an optical transient absorption spectroscopy experiment. We attribute the strain to the piezoelectric effect due to screening of the depolarization field by laser induced carriers. A strong film thickness dependence of strain and carrier relaxation is also observed, revealing the role of the carrier transport in determining the structural and carrier dynamics in complex oxide thin films.
Whether ethics is too important to be left to the experts or so important that it must be is an age-old question. The emergence of clinical ethicists raises it again, as a question about professionalism. What role clinical ethicists should play in healthcare decision making – teacher, mediator, or consultant – is a question that has generated considerable debate but no consensus.
The primary motivation for China's economic reforms was to increase economic growth and raise living standards after nearly twenty years of stagnation. Given the move to more market-based income determination, the reforms had the potential to conflict with inherited egalitarian-motivated socialist institutions and rhetoric. To what extent have the reforms led to widening inequality? Who have been the winners and losers? Have the reductions in poverty that accompanied growth been sufficient to alleviate concerns over inequality? Do increases in inequality threaten the long-run sustainability of the reforms? Are there identifiable patterns in the evolution of the income distribution that suggest potential policy responses?
The objective of this chapter is to document the evolution of inequality, and the income distribution more generally, during the reform period and where possible to draw conclusions concerning the role that transition has played in increasing inequality. The centerpiece of our chapter is the assembly of three cross-section data sets that allow a relatively consistent calculation of inequality from the mid-1980s onward. It turns out that establishing “first-order” facts about Chinese inequality is quite difficult and that conclusions hinge on mundane (but important) issues of measurement and data quality. In this regard, it is unfortunate that much of the household-level survey data collected annually by China's National Bureau of Statistics (NBS) remains outside the public domain.
Using data from the China Urban Labour Survey conducted in five large Chinese cities at year end 2001, we quantify the nature and magnitude of shocks to employment and worker benefits during the period of economic restructuring from 1996 to 2001, and evaluate the extent to which adversely affected urban workers had access to public and private assistance. Employment shocks were large and widespread, and were particularly hard on older workers and women. During the period of economic restructuring, unemployment reached double figures in all sample cities and labour force participation declined by 8.9 per cent. Urban residents faced modest levels of wage and pension arrears, and sharp declines in health benefits. Public assistance programmes for dislocated workers had limited coverage, with most job-leavers relying upon private assistance to support consumption, mainly from other household members.
The ability of a poly(phenylene ethynylene) conjugated polyelectrolyte to detect a small molecule quencher in the solid state was assessed by coating the polymer onto a plastic-backed, non-fluorescent, silica-gel TLC plate. The efficiency of the emission quenching process was quantified by noting the resulting loss of emission with increasing quencher concentration, in accordance with the Stern-Volmer relation. The emission quenching induced by the addition of the analyte occurred with Stern-Volmer constants in the range of 103 M−1.
We introduce a property formally weaker than weak uniform rotundity, which we call equatorial weak uniform rotundity. We show that an equatorially weakly uniformly rotund norm need not be weakly locally uniformly rotund. Nevertheless, we show that an equatorially weakly uniformly rotund Banach space is an Asplund space.
One of the striking outcomes of China's economic reforms is the emergence of inter-regional labour markets as rural workers have poured into the nation's urban and rural economies. Policy makers in China, as elsewhere in the world, have treated the inter-regional migrant labour force with ambiguity. Migration may increase efficiency, contribute to poverty reduction and make China's economy more competitive, but leaders fear the congestion, social unrest and loss of political control which might accompany an increasingly mobile labour force.
The effects of lithium on vascular development were examined using the chick embryo area vasculosa in
shell-less culture as an experimental model. Embryos were explanted after 48 h in ovo and LiCl (50, 100, 150
and 200 μg in 10 μl water) was applied to the centre of the blastodisc. Controls were untreated or given
equimolar amounts of NaCl. At 24 h and 48 h after treatment, untreated and NaCl controls were identical,
having well developed extraembryonic vessels. At doses of 100 μg and greater, LiCl significantly inhibited
normal vascular development and expansion of the area vasculosa in the majority of explants. In many
specimens blood islands continued to form but their assembly into primitive vessels was prevented,
indicating that lithium affects the mechanism regulating the assembly of vascular endothelium. At the same
time the embryos were alive but retarded in development compared with controls. When LiCl (150 μg) was
applied to cultures explanted after 72 h in ovo (when the primary vascular network had already formed
through vasculogenesis) no adverse effects were seen. This suggests that lithium affects vasculogenesis but
not angiogenesis. Treatment with myo-inositol completely reversed the effects of lithium in a time dependent
manner indicating that the phosphatidylinositol second messenger cycle may be involved in the cellular
events of vasculogenesis. Finally the results of this study show that the yolk sac vasculature is particularly
vulnerable to lithium and the consequent effects of this interference on embryonic development are
discussed.
We study a nonsmooth extension of Gateaux differentiability satisfying a directional Hölder condition. In particular, we show that a Banach space is an Asplund space if it has an equivalent norm with a directionally Hölder right-hand derivative at each point of its sphere.
A method has been developed for producing large area single-crystal diamond plates, suitable for optical and electronic applications. It starts with orienting and closely packing a set of diamond seed crystals with (001) top faces. This assembly, or mosaic, is then joined by a single-crystal overgrowth using a CVD process. A number of assembling techniques have been tested for compatibility with homoepitaxial diamond growth by hot filament assisted CVD and/or growth and etching by the acetylene-oxygen combustion flame. Furthermore a two-step process is described. First an initial layer (20-50/μm) is deposited by hot filament assisted CVD at a low growth rate in order to bridge the gap between the seeds. Subsequently the fast growth rate of the acetylene-oxygen combustion flame is employed to increase the layer thickness (>250,μm). It was found that both the basic mosaic process as well as the two step process can produce a single-crystal diamond layer on top of mosaics consisting of seed crystals with well aligned crystallographic directions. The width of the gaps between the seed crystals (up to 25 μm) was found to be less critical, while the orientation of the side faces and the direction of the misorientation (i.e. the step flow direction) seem not to effect the successful overgrowth. Apart from the alignment of the seed crystals the most severe problem, which has to be overcome in order to obtain one single-crystal overgrowth, is the occurrence of penetration twins in the joint regions. The largest mosaic structure -up to now- overgrown by CVD consists of seven seed crystals and has a surface area slightly in excess of 1 cm2