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Hypertrophic cardiomyopathy is an autosomal dominant hereditary disease characterised by left ventricular asymmetry hypertrophy. However, our knowledge of the genetic background in hypertrophic cardiomyopathy cases is limited. Here, we aimed to evaluate pathogenic gene mutations in a family with high-risk hypertrophic cardiomyopathy and analyse the genotype/phenotype relationships in this family.
The proband, her parents, and her niece underwent whole-exome sequencing, and the genotypes of family members were identified using Sanger sequencing. mRNA expression was detected using reverse transcription sequencing. Structural impairments were predicted by homologous modelling. A family survey was conducted for patients with positive results to obtain information on general clinical symptoms, electrocardiography, ambulatory electrocardiography, echocardiography, and 3.0T cardiac magnetic resonance findings. Regular follow-up was performed for up to 6 months.
Five family members, including the proband, carried a cleavage site mutation in the MYBPC3 gene (c.2737+1 (IVS26) G>T), causing exon 26 of the MYBPC3 gene transcript to be skipped and leading to truncation of cardiac myosin-binding protein C. Family survey showed that the earliest onset age was 13 years old, and three people had died suddenly at less than 40 years old. Three pathogenic gene carriers were diagnosed with hypertrophic cardiomyopathy, and all showed severe ventricular septal hypertrophy.
The c.2737+1 (IVS26) G>T mutation in the MYBPC3 gene led to exon 26 skipping, thereby affecting the structure and function of cardiac myosin-binding protein C and leading to severe ventricular hypertrophy and sudden death.
As a wide-reaching institutional reform, China's fiscal decentralization was launched in the early 1980s to encourage provincial economic growth by granting more financial autonomy to provincial governments. In this paper, the impact of fiscal decentralization on China's environmental quality is investigated both theoretically and empirically. A neoclassical model is developed based on the primary characteristics of China's fiscal decentralization. Using provincial panel data for the period 1995-2015, a two-equation regression model is employed to empirically verify the three propositions of the theoretical model: (1) there exists an inverted-U shaped relationship between fiscal decentralization and GDP per capita; (2) fiscal decentralization is positively related to GDP per capita at the steady state; (3) there is an inverted-U shaped Environmental Kuznets Curve relationship between pollution emissions and economic growth.
Echinococcus granulosus sensu stricto (s.s.), Echinococcus multilocularis and Echinococcus canadensis are the common causes of human echinococcosis in China. An accurate species identification tool for human echinococcosis is needed as the treatments and prognosis are different among species. The present work demonstrates a method for the simultaneous detection of these three Echinococcus species based on multiplex polymerase chain reaction (mPCR). Specific primers of this mPCR were designed based on the mitochondrial genes and determined by extensive tests. The method can successfully detect either separated or mixed target species, and generate expected amplicons of distinct size for each species. Sensitivity of the method was tested by serially diluted DNA, showing a detection threshold as less as 0.32 pg for both E. granulosus s.s. and E. canadensis, and 1.6 pg for E. multilocularis. Specificity assessed against 18 other parasites was found to be 100% except weakly cross-react with E. shiquicus. The assay was additionally applied to 69 echinococcosis patients and 38 healthy persons, confirming the high reliability of the method. Thus, the mPCR described here has high application potential for clinical identification purposes, and can further provide a useful tool for evaluation of serology and imaging method.
Asia Minor bluegrass (Polypogon fugax Nees ex Steud.) is a problem grass weed of winter crops in China, where a population has become resistant to aryloxyphenoxypropionate (APP) herbicides. The mechanism of resistance is due to an Ile-2041-Asn mutation of the ACCase gene. Screen house experiments were conducted to study the growth, fecundity characteristics, and competitive ability of this aryloxyphenoxypropionate-resistant (APP-R) biotype compared with a susceptible (APP-S) biotype. When grown under noncompetitive conditions, the APP-R P. fugax developed more rapidly than the APP-S plants, with earlier tiller and panicle emergence and seed shedding; the APP-R P. fugax set seeds nearly 12 d earlier than the APP-S biotype. APP-R and APP-S biotypes had similar aboveground dry weight before the flowering stage. Fecundity of the APP-R biotype was similar to the APP-S biotype (8.57 g seeds plant−1 and 0.17 g seeds panicle−1 versus 8.22 g seeds plant−1 and 0.13 g seeds panicle−1, respectively). Ultimately, the relatively slower-developing APP-S P. fugax had 50% more shoot dry weight than the APP-R plants. Relative competitiveness among the APP-R and APP-S P. fugax biotypes was investigated through replacement series experiments. No difference in competitive ability was measured between APP-R and APP-S biotypes on the basis of shoot dry weight before the tillering stage. These results indicate that there is no apparent fitness penalty for the APP-R P. fugax. The shorter growth cycle of APP-R with no apparent fitness penalty suggests that growers will need begin weed control earlier and possibly include vegetative crops with an even shorter growth cycle in their rotations.
Maternal one-carbon metabolism during pregnancy is crucial for fetal development and programming by DNA methylation. However, evidence on one-carbon biomarkers other than folate is lacking. We, therefore, investigated whether maternal plasma methyl donors, that is, choline, betaine and methionine, are associated with birth outcomes. Blood samples were obtained from 115 women during gestation (median 26·3 weeks, 90 % range 22·7–33·0 weeks). Plasma choline, betaine, methionine and dimethylglycine were measured using HPLC-tandem MS. Multivariate linear and logistic regression models were used to estimate the association between plasma biomarkers and birth weight, birth length, the risk of small-for-gestational-age and large-for-gestational-age (LGA). Higher level of maternal betaine was associated with lower birth weight (–130·3 (95 % CI –244·8, –15·9) per 1 sd increment for log-transformed betaine). Higher maternal methionine was associated with lower risk of LGA, and adjusted OR, with 95 % CI for 1 sd increase in methionine concentration was 0·44 (95 % CI 0·21, 0·89). Stratified analyses according to infant sex or maternal plasma homocysteine status showed that reduction in birth weight in relation to maternal betaine was only limited to male infants or to who had higher maternal homocysteine status (≥5·1 µmol/l). Higher maternal betaine status was associated with reduced birth weight. Maternal methionine was inversely associated with LGA risk. These findings are needed to be replicated in future larger studies.
The excessive use of plastic, especially polystyrene (PS), has caused serious environmental pollution. The efficient utilization of plastics and the conversion of plastics into value-added carbon materials are the concerns of researchers. Herein, we propose novel “pyrolysis–deposition” method to convert one popular plastic substance, PS, into ordered mesoporous carbons (OMCs). During the synthesis process, PS is pyrolyzed into small organic gases under high temperature, which is then adsorbed through capillary adsorption into the mesoporous of SBA-15 in the presence of catalyst. The obtained OMCs have high specific surface area, uniform pore size, and ordered pore structure. The OMCs exhibit specific capacitance of 118 F/g at a current density of 0.2 A/g and electrochemical stability of 87.2% at a current density of 2 A/g after 5000 cycles. The pyrolysis–deposition strategy provides a new idea to convert waste plastics into high-performance carbon materials for electrochemical applications.
The accounting information of a firm is analogous to the characteristics of an organism that contain biological information that influences decisions; such characteristics result from organizational routines (genes). Organizational routines result from organizational learning, and learning from an associated company is an efficient approach for a new venture to establish routines. The study results revealed that the subsidiaries inherited routines from the parent companies related to financial ratios, so we suggest that people should judge the adequacy of a firm’s financial situation by not only referring to the standard of its industry but also to its parent company.
Several studies have suggested that higher carotenoid levels may be beneficial for atherosclerosis patients, but few studies have examined this relationship in the Chinese population. This cross-sectional study examined the association between the levels of carotenoids in diet and serum and carotid intima–media thickness (IMT) in Chinese adults aged 50–75 years in Guangzhou, China. Dietary intake was assessed using a FFQ. HPLC was used to assay the serum concentrations of α-carotene, β-carotene, lutein+zeaxanthin, β-cryptoxanthin and lycopene. The IMT at the common carotid artery (CCA) and bifurcation of the carotid artery was measured by B-mode ultrasound. A total of 3707 and 2947 participants were included in the analyses of dietary and serum carotenoids. After adjustment for demographic, socio-economic and lifestyle factors, all the serum carotenoids levels except lycopene were found to be inversely associated with the IMT at the CCA and bifurcation (Ptrend<0·001 to 0·013) in both men and women. The absolute mean differences in the IMT between the subjects in the extreme quartiles of serum carotenoid levels were 0·034 mm (α-carotene), 0·037 mm (β-carotene), 0·032 mm (lutein+zeaxanthin), 0·030 mm (β-cryptoxanthin), 0·015 mm (lycopene) and 0·035 mm (total carotenoids) at the CCA; the corresponding values were 0·025, 0·053 0·043, 0·050, 0·011 and 0·042 mm at the bifurcation. The favourable associations were also observed between dietary carotenoids (except lycopene) and the CCA IMT. In conclusion, elevated carotenoid levels in diet and serum are associated with lower carotid IMT values (particular at the CCA) in Chinese adults.
Apathy is a condition characterized by a lack of motivation that manifests in emotional, behavioral, and cognitive domains. Although previous studies have indicated that apathy is associated with frontal lesions, few studies have focused on the different subdomains of apathy, and no in vivo human biochemical data have been obtained to examine the neurochemical changes related to apathy in patients with Alzheimer's disease (AD). Thus, we investigated the frontal neurochemical alterations related to apathy among patients with AD using proton magnetic resonance spectroscopy (1H MRS).
Apathy was assessed through the Apathy Evaluation Scale (AES). 1H MRS was performed to measure neurochemical metabolite levels in the anterior cingulate region and right orbitofrontal region. Associations between neurochemical metabolites and the total score and subscores of each domain of the AES were analyzed.
Altogether, 36 patients completed the study. Patients with lower N-acetylaspartate/creatine ratios (NAA/Cr) in the anterior cingulate region demonstrated higher total apathy scores (β = −0.56, p = 0.003) with adjustments for age, gender, educational level, dementia severity, and depression severity. In a further analysis, a lower NAA/Cr in the anterior cingulate region was associated with all subdomains of apathy, including cognition (β = −0.43, p = 0.028), behavior (β = −0.55, p = 0.002), and emotion (β = −0.50, p = 0.005). No statistically significant associations were discovered in the right orbitofrontal region.
Our results suggest that apathy, in each of its cognitive, behavioral, or emotional subdomains is associated with brain neurochemical alterations in the anterior cingulate region. Abnormal neuronal integrity over the anterior cingulate cortex may exhibit a central role in causing all aspects of apathy in patients with AD.
This article outlines the evolution of a rescue team in responding to adenovirus prevention with a deployable field hospital. The local governments mobilized a shelter hospital and a rescue team consisting of 59 members to assist with rescue and response efforts after an epidemic outbreak of adenovirus. We describe and evaluate the challenges of preparing for deployment, field hospital maintenance, treatment mode, and primary treatment methods. The field hospital established at the rescue scene consisted of a medical command vehicle, a computed tomography shelter, an X-ray shelter, a special laboratory shelter, an oxygen and electricity supply vehicle, and epidemic prevention and protection equipment. The rescue team comprised paramedics, physicians, X-ray technicians, respiratory therapists, and logistical personnel. In 22 days, more than 3000 patients with suspected adenovirus infection underwent initial examinations. All patients were properly treated, and no deaths occurred. After emergency measures were implemented, the spread of adenovirus was eventually controlled. An emergency involving infectious diseases in less-developed regions demands the rapid development of a field facility with specialized medical personnel when local hospital facilities are either unavailable or unusable. An appropriate and detailed prearranged action plan is important for infectious diseases prevention. (Disaster Med Public Health Preparedness. 2018;12:109–114)
The metamorphic responses of mussel (Mytilus coruscus) larvae to pharmacological agents affecting G proteins and the adenylate cyclase/cyclic AMP (AC/cAMP) pathway were examined in the laboratory. The G protein activators guanosine 5′-[β,γ-imido]triphosphate trisodium salt hydrate and guanosine 5′-[γ-thio]triphosphate tetralithium salt only induced larval metamorphosis in continuous exposure assays, and the G protein inhibitor guanosine 5′-[β-thio]diphosphate trilithium salt did not exhibit inducing activity. The non-specific phosphodiesterase inhibitor theophylline and the cAMP-specific phosphodiesterase IV inhibitor 4-(3-Butoxy-4-methoxybenzyl)imidazolidin-2-one exhibited inducing activity, while the non-specific phosphodiesterase inhibitor 3-Isobutyl-1-methylxanthine only showed inducing activity at 10−4 M in continuous exposure assays. The cyclic nucleotide analogue N6,2′-O-Dibutyryladenosine 3′,5′-cyclic monophosphate sodium salt did not exhibit significant inducing activity. Both the adenylate cyclase activator forskolin and the adenylate cyclase inhibitor nitroimidazole exhibited inducing activity at 10−4 to 10−3 M concentrations in continuous exposure assays. Among these tested agents, the adenylate cyclase inhibitor (±)-miconazole nitrate salt showed the most promising inducing effect. The present results indicate that G protein-coupled receptors and signal transduction by AC/cAMP pathway could mediate metamorphosis of larvae in this species.
Horizontal convection, with a focus on the essential physics it can disclose for the oceanic overturning circulation, is studied in an annular gap where differential heating is applied along the bottom. Based on the three-dimensional distribution of both velocity and temperature fields, we propose that in addition to differential buoyancy forcing and diapycnal mixing, potential vorticity (PV) can also be a limiting factor for the overturning circulation by imposing a constraint on the meridional flow. With rotation, flow has to be along PV contours excepting that effects of diffusion or friction exist to break the PV constraint. In the axisymmetric rotating experiment, the flow along isotherms is to zero-order azimuthal, due to the fact that PV varies only along the radial direction. Consequently, the radial flow appears at the next order with much smaller amplitude, leading to a much weaker overturning circulation compared with that of the non-rotating experiment. In the presence of a full radial barrier, the annular gap can be viewed as a four-sided domain with its northern boundary along the inner cylinder, and the meridional flow shows remarkable inhomogeneity in space. In the absence of any background PV gradient, the flow field equilibrates itself to a state where the PV contours above the bottom boundary layer are largely radial, particularly in a sub-region near the western boundary, allowing a relatively strong radial geostrophic flow and greatly strengthening the overturning circulation from the case without the barrier.
Gauged river flow records from China generally span only a few decades, which hampers the detection of long-term, decadal- to centennial-scale cycles and trends in streamflow variability. New and updated tree-ring chronologies help reconstructed the water-year (October–September) streamflow for the Aksu River, which is an important river at the edge of the Taklimakan Desert that drains into the Tarim Basin. The reconstruction dates back to 1692 and has an adjusted r2 of 0.61 (1957–2006). Based on frequency, intensity and duration of droughts and pluvial events, the lowest streamflows occurred in the 1920s. Since then streamflow has continuously increased, and was exceptionally rapidly after the 1960s, until today. The start and end of the 20th century to the present were the highest streamflow periods. The mid-20th century was the longest and driest period over the past 300 yr. The reconstructed streamflow series has a strong positive correlation with the North Atlantic Oscillation Index. Changes in mid-latitude circulation patterns influencing precipitation may have indirectly resulted in streamflow variations along the Aksu River over the past 300 yr. The rapid increase and the exceptional streamflows of the 1960s are likely linked with global warming and mid-latitude atmospheric circulation changes.
This is a case-control study to investigate the prevalence, characteristics, and risk factors of pain in patients with Parkinson's disease (PD).
A total of 200 PD patients from eastern China were enrolled in our study. Accordingly, 200 healthy elderly adults were recruited as controls. The characteristics of pain were collected by using the Visual Analog Scale, Brief Pain Inventory (BPI), SF-36 Bodily Pain Scale, Unified Parkinson's Disease Rating Scale, Hoehn–Yahr Scale (H-Y), Hamilton Depression Scale, and Leeds Assessment of Neuropathic Symptoms and Signs.
Of the 200 PD patients, pain was complained by 106 patients (53%). According to the SF-36 Bodily Pain Scale, pain morbidity in PD patients was significantly higher than in the control group. The average pain during last 24 h measured by the BPI was 2.67. About 76% of PD patients were found to have one pain type, 21.7% were having two pain types, and 1.9% had three pain types. Further, 69.8% of these patients were presented with musculoskeletal pain, 4.7% with dystonic pain, 22.6% with radicular-neuropathic pain, 20.8% with central neuropathic pain, and 9.4% with akathisia pain. The onset age and depression were the most significant predictors of pain in PD patients (p < 0.05). However, there was no significant association between pain and gender, age, disease duration, or severity of the disease. Only 5.7% of PD patients with pain received treatment in this study.
Pain is frequent and disabling, independent of demographic and clinical variables, and is significantly more common in PD patients.