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ABSTRACT Objectives The calculation of left ventricular mass varies in different studies, and reference values of the voltage‐to‐mass ratio for diagnosing cardiac amyloidosis (CA) are lacking. This study aimed to determine the value of the voltage‐to‐mass ratio in diagnosing CA and provide an optimal cut‐off value for different calculation methods. Methods We reviewed the electrocardiograms and echocardiograms of 213 consecutive biopsy‐proven CA patients, 236 hypertrophic cardiomyopathy (HCM) patients, 100 hypertensive heart disease patients, and 181 healthy controls. Left ventricular mass was calculated using linear and cross‐sectional area (CSA) methods. The voltage‐to‐mass ratios were compared between the CA group and other groups. The voltage‐to‐mass ratio obtained was used to build multivariate logistic regression models that predicted the log odds of developing CA. Results The CA group had a significantly lower voltage‐to‐mass ratio than the HCM, hypertensive heart disease, and healthy control groups. The voltage‐to‐mass ratio was an independent factor significantly associated with the CA diagnosis after adjusting for baseline characteristics. Linear and CSA methods yielded areas under the ROC curve of 0.86 and 0.90, respectively. Using the CSA method, the optimal cut‐off was 16.42 mV/mm2/m2, with 89.0% sensitivity and 80.8% specificity. Conclusion The voltage‐to‐mass ratio could differentiate patients with CA, HCM, and hypertensive heart disease from healthy controls, potentially providing an accurate and non‐invasive alternative to current expensive and invasive diagnostic techniques.

Objection: The rapid advancement of artificial intelligence has brought significant breakthroughs to various medical disciplines，This study aimed to compare perioperative factors and postoperative hip function recovery in primary total hip arthroplasty (THA)by evaluating the use of an artificial intelligence (AI) preoperative planning system versus traditional two-dimensional X-ray planning. Patients and methods: A total of 45 eligible patients underwent primary THA at Beijing Shijitan Hospital, Capital Medical University, between July 2022 and August 2022. The patients were randomly assigned to either the experimental group (n = 19) or the control group (n = 26). The experimental group utilized AI planning, while the control group employed traditional two-dimensional X-ray planning. Statistical analysis was performed to compare the accuracy of prosthesis prediction, operation time, intraoperative blood loss, frequency of intraoperative model testing, length of hospital stay, postoperative imaging data, and postoperative hip function scores. These comparisons were made to assess the effects of different preoperative planning methods on perioperative and postoperative hip function recovery. Results: The accuracy of preoperative planning for the acetabular and femoral sides in AI-assisted total hip arthroplasty was 84.2 % and 89.5 %, respectively, which was significantly better than that of the traditional two-dimensional X-ray planning group (P

The Xiashu loess is an important aeolian deposit in eastern China. Research on the provenance of the Xiashu loess, located along the lower reaches of the Yangtze River, has entered into a new stage, compelling us to review the previous research findings in order to provide direction for future research. In this study, we enumerate three different viewpoints regarding the major sources of the loess in eastern China, including distant sources, proximal sources, and mixed sources. We also discuss disturbance factors, methods, and research on the provenance of the Xiashu loess. Previous research has showed that, on the basis of geochronology and meticulous grain grading data, good results as to the loess’ provenance can be obtained using immobile geochemical index tracers that are not affected by chemical weathering or grain size effects. Examples include detrital zircon U-Pb age spectra, major and trace element ratios, stable isotopic composition, and slowly weatherable minerals. However, differences in the data do exist, e.g., for the immobile geochemical provenance tracing indexes of the loess. Also still under debate is the age of the lower boundary of the loess. Therefore, exploring the spatial-temporal variations of the provenance, along with the coupling relationship between the paleoclimate environment evolution and the onset of the loess accumulation, should be comprehensively considered in future research.

Abstract Background and aims The 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guideline has recently revised the hemodynamic definition of pulmonary arterial hypertension. However, there is currently limited research on the prognosis and treatment of system lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH) patients that have been reclassified by the new hemodynamic definition. This study aims to analyze the prognosis of newly reclassified SLE-PAH patients and provide recommendations for the management strategy. Methods This retrospective study analyzed records of 236 SLE-PAH patients who visited Peking Union Medical College Hospital (PUMCH) from 2011 to 2023, among whom 22 patients were reclassified into mild SLE-PAH (mean pulmonary arterial pressure (mPAP) of 21–24 mmHg, pulmonary vascular resistance (PVR) of 2–3 WU, and PAWP ≤ 15 mmHg) according to the guidelines and 14 were defined as unclassified SLE-PAH patients (mPAP 21–24 mmHg and PVR ≤ 2 WU). The prognosis was compared among mild SLE-PAH, unclassified SLE-PH, and conventional SLE-PAH patients (mPAP ≥ 25 mmHg and PVR > 3WU). Besides, the effectiveness of pulmonary arterial hypertension (PAH)-specific therapy was evaluated in mild SLE-PAH patients. Results Those mild SLE-PAH patients had significantly longer progression-free time than the conventional SLE-PAH patients. Among the mild SLE-PAH patients, 4 did not receive PAH-specific therapy and had a similar prognosis as patients not receiving specific therapy. Conclusions This study supports the revised hemodynamic definition of SLE-PAH in the 2022 ESC/ERS guideline. Those mild and unclassified SLE-PH patients had a better prognosis, demonstrating the possibility and significance of early diagnosis and intervention for SLE-PAH. This study also proposed a hypothesis that IIT against SLE might be sufficient for those reclassified SLE-PAH patients.

ABSTRACT Purpose: It has been reported that exhaustive exercise (EE) causes myocyte injury, and eventually damages the function of the myocardia. Albiflorin (AF) has anti-inflammatory, antioxidant, and anti-apoptosis effects. In this study, we determined whether AF could mitigate the EE-induced myocardial injury and research the potential mechanisms. Methods: The rat model of EE was built by forced treadmill running method. Rats were intraperitoneally injected with AF before EE once daily for one week. The relative factors levels were examined by commercial kits. The apoptosis was appraised using a TdT-mediated dUTP nick end labeling assay kit. The ACSL4, GPX4, Nrf2, pAKT/AKT, and HO-1 contents were assessed by western blot. Results: AF lessened EE-induced cardiac myocytes ischemic/hypoxic injury and reduced the contents of myocardial injury biomarkers in the serum. AF lessened EE-induced cardiac myocyte apoptosis, inflammatory response, oxidative stress, and ferroptosis in myocardial tissues. However, the influences of AF were overturned by the co-treatment of AF and LY294002. AF activated the AKT/Nrf2/HO-1 signaling pathway in myocardial tissues in vivo. Conclusions: AF could curb cardiac myocytes ferroptosis, thus diminishing the EE-induced myocardial injury through activating the AKT/Nrf2/HO-1 cascade.

Abstract Background Public health workers are a crucial part of the health workforce, particularly during the coronavirus disease (COVID-19) pandemic. They play an important role in achieving universal health coverage and sustainable development goals. Human resources in public health in China are in short supply, their distribution is unequal, and their turnover rate is high. A discrete choice experiment (DCE) was applied to investigate preventive medicine students’ preferred job choice criteria and trends in trade-offs by calculating the marginal rate of substitution between these criteria. This study identified the properties of jobs primarily selected by preventive medicine students and estimated the monetary value of each attribute. Methods Based on discussions and in-depth interviews with preventive medicine students and a literature review, we developed a DCE that assessed how students’ stated preferences for a certain choice were influenced by several job attributes, including location, salary, bianzhi, career development opportunities, working environment, and workload. We applied this DCE to preventive medicine students in Shandong Province, China, using a brief, structured questionnaire. Conditional logit models were used to estimate the utility of each job’s attributes. Willingness to pay (WTP) was estimated as the ratio of the value of the coefficient of interest to the negative value of the cost attribute. Results A total of 307 respondents completed the questionnaire, and 261 passed the internal consistency test. All the attributes were statistically significant. Career development opportunities and work locations were the most important factors for the respondents. Preference heterogeneity existed among respondents, e.g., 3-year medical education college students placed a higher value on jobs with bianzhi compared to 5-year medical education college students. Furthermore, rural students’ WTP for a job located in the county or city is much lower than that of urban students. Conclusions The heterogeneity of attributes indicates the complexity of job preferences. Monetary and nonmonetary job characteristics significantly influenced the job preferences of preventive medicine students in China. A more effective policy intervention to attract graduates to work in rural areas should consider both job incentives and the backgrounds of preventive medicine graduates.

Abstract Background Pulmonary arterial hypertension is a major cause of death in systemic lupus erythematosus, but there are no tools specialized for predicting survival in systemic lupus erythematosus-associated pulmonary arterial hypertension. Research question To develop a practical model for predicting long-term prognosis in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension. Methods A prognostic model was developed from a multicenter, longitudinal national cohort of consecutively evaluated patients with systemic lupus erythematosus-associated pulmonary arterial hypertension. The study was conducted between November 2006 and February 2020. All-cause death was defined as the endpoint. Cox regression and least absolute shrinkage and selection operators were used to fit the model. Internal validation of the model was assessed by discrimination and calibration using bootstrapping. Results Of 310 patients included in the study, 81 (26.1%) died within a median follow-up of 5.94 years (interquartile range 4.67–7.46). The final prognostic model included eight variables: modified World Health Organization functional class, 6-min walking distance, pulmonary vascular resistance, estimated glomerular filtration rate, thrombocytopenia, mild interstitial lung disease, N-terminal pro-brain natriuretic peptide/brain natriuretic peptide level, and direct bilirubin level. A 5-year death probability predictive algorithm was established and validated using the C-index (0.77) and a satisfactory calibration curve. Risk stratification was performed based on the predicted probability to improve clinical decision-making. Conclusions This new risk stratification model for systemic lupus erythematosus-associated pulmonary arterial hypertension may provide individualized prognostic probability using readily obtained clinical risk factors. External validation is required to demonstrate the accuracy of this model's predictions in diverse patient populations.

Not available.

This study considered Shilin World Geopark as the research object and constructed a landscape ecological risk assessment model based on the landscape pattern index by using remote sensing image data during five periods between 2000 and 2020. In addition, it analyzed the spatial and temporal changes of landscape ecological risk in the region. Spatial autocorrelation analysis was utilized to study spatial differences in the landscape ecological risk in the park. The results showed that during the study period, (1) cultivated land, forest land, and rocky desertification land were the main landscape types, different landscape types differed, and the area of rocky desertification land and building land increased by 37.47 km2 and 14.29 km2, respectively, while the area of cultivated land and grassland decreased significantly, with changes of 34.11 km2 and 18.67 km2; (2) landscape ecological risk of the park showed significant spatial differences, the ‘high–high’ risk areas have been concentrated mainly in the central and northern parts of the park, the ‘low–low’ risk areas have been concentrated in the central part and the southwest-southeast area of the park; and (3) landscape ecological risk of the geopark has been increasing, with the degree of landscape ecological risk being spatially positively correlated. The results of the study are of great significance for maintaining ecosystem health of the Shilin World Geopark and optimizing the ecological risk management of the park.

With rapid urbanization, retrieving information about residential complexes in a timely manner is essential for urban planning. To develop efficiency and accuracy of building extraction in residential complexes, a Segment Anything Model-based residential building instance segmentation method with an automated prompt generator was proposed combining LiDAR data and VHR remote sensing images in this study. Three key steps are included in this method: approximate footprint detection using LiDAR data, automatic prompt generation for the SAM, and residential building footprint extraction. By applying this method, residential building footprints were extracted in Pukou District, Nanjing, Jiangsu Province. Based on this, a comprehensive assessment model was constructed to systematically evaluate the spatial layout of urban complexes using six dimensions of assessment indicators. The results showed the following: (1) The proposed method was used to effectively extract residential building footprints. (2) The residential complexes in the study area were classified into four levels. The numbers of complexes classified as Excellent, Good, Average, and Poor were 10, 29, 16, and 1, respectively. Residential complexes of different levels exhibited varying spatial layouts and building distributions. The results provide a visual representation of the spatial distribution of residential complexes that belong to different levels within the study area, aiding in urban planning.

Background: Long-term prognosis of dilated cardiomyopathy (DCM) in the Chinese population is lacking, and the left ventricular (LV) hypertrabeculation phenotype usually overlaps with DCM. Objectives: The study aims to investigate whether the presence of the LV hypertrabeculation phenotype confers additional adverse prognostic information for DCM patients. Methods: We retrospectively reviewed all DCM patients (≥18 years of age at diagnosis) hospitalized in the Peking Union Medical College Hospital between September 2002 and September 2022. The eligible patients were divided into two groups based on echocardiography at diagnosis: the isolated DCM (n = 353), and DCM with the LV hypertrabeculation phenotype (n = 97). The primary endpoint was major adverse cardiac events (MACEs), and multivariate Cox hazards regression models were used to compare the endpoints between the two groups. Results: During a mean follow-up time of 4.6 years, there was no significant difference in the primary endpoint between the isolated DCM and DCM with the LV hypertrabeculation phenotype (p = 0.19). The risk of MACEs in the first 5 years was significantly higher in DCM with the LV hypertrabeculation phenotype than isolated DCM (adjusted HR [95%CI]: 1.83 [1.21–2.77]) and after 5 years the effect of the LV hypertrabeculation phenotype as a prognostic attenuated. Subgroup analysis found a significant interaction for the incidence of MACEs between sex and DCM subtypes (p for interaction = 0.01). Conclusions: DCM with LV hypertrabeculation phenotypes had a higher early (first 5 years) risk of MACEs. For males, the presence of LV hypertrabeculation phenotypes might be an important clue for identifying high-risk DCM patients.

Background: Amyloid light-chain (AL) amyloidosis is a multisystem disorder, with cardiac amyloid infiltration being a prevalent manifestation. This study aimed to explore the prognostic value of galectin-3 (Gal-3), a soluble marker associated with fibrosis, inflammation, heart failure, and kidney injury, in patients with cardiac AL amyloidosis. Methods: A total of 60 patients who were diagnosed with cardiac AL amyloidosis from January 2015 to May 2018 were enrolled. The prognostic value of Gal-3 was assessed. Receiver operating characteristic (ROC) curves were used to evaluate the predictive accuracy of Gal-3. A Gal-3 cut-off value was identified to predict survival rates. Results: The ROC curves demonstrated a moderate predictive accuracy of Gal-3 for 0.5- and 5-year survival, with area under the curve (AUC) values of 0.722 and 0.788, respectively. A Gal-3 cut-off value of 15.154 ng/mL was found to predict survival. Kaplan–Meier survival analysis revealed a significant difference in mean overall survival between patients with Gal-3 levels below and above the established cut-off (69.2 months versus 42.1 months, respectively; p = 0.036). Multivariate analysis confirmed that Gal-3 > 15.154 ng/mL remained an independent predictor of survival (HR 2.451, 95% CI 1.017–5.910, p = 0.046). Conclusions: This study suggests that Gal-3 holds independent prognostic value for survival in patients with cardiac AL amyloidosis. Gal-3 could potentially enhance the prognostic capabilities of the current soluble markers, thereby improving the management of cardiac AL amyloidosis. However, further validation in larger prospective studies is warranted.

Defect inspection of existing buildings is receiving increasing attention for digitalization transfer in the construction industry. The development of drone technology and artificial intelligence has provided powerful tools for defect inspection of buildings. However, integrating defect inspection information detected from UAV images into semantically rich building information modeling (BIM) is still challenging work due to the low defect detection accuracy and the coordinate difference between UAV images and BIM models. In this paper, a deep learning-based method coupled with transfer learning is used to detect defects accurately; and a texture mapping-based defect parameter extraction method is proposed to achieve the mapping from the image U-V coordinate system to the BIM project coordinate system. The defects are projected onto the surface of the BIM model to enrich a surface defect-extended BIM (SDE-BIM). The proposed method was validated in a defect information modeling experiment involving the No. 36 teaching building of Nantong University. The results demonstrate that the methods are widely applicable to various building inspection tasks.

Human cartilage tissue can be categorized into three types: hyaline cartilage, elastic cartilage and fibrocartilage. Each type of cartilage tissue possesses unique properties and functions, which presents a significant challenge for the regeneration and repair of damaged tissue. Bionics is a discipline in which humans study and imitate nature. A bionic strategy based on comprehensive knowledge of the anatomy and histology of human cartilage is expected to contribute to fundamental study of core elements of tissue repair. Moreover, as a novel tissue-engineered technology, 3D bioprinting has the distinctive advantage of the rapid and precise construction of targeted models. Thus, by selecting suitable materials, cells and cytokines, and by leveraging advanced printing technology and bionic concepts, it becomes possible to simultaneously realize multiple beneficial properties and achieve improved tissue repair. This article provides an overview of key elements involved in the combination of 3D bioprinting and bionic strategies, with a particular focus on recent advances in mimicking different types of cartilage tissue.

Abstract Background In recent years, there has been significant research progress on in situ articular cartilage (AC) tissue engineering with endogenous stem cells, which uses biological materials or bioactive factors to improve the regeneration microenvironment and recruit more endogenous stem cells from the joint cavity to the defect area to promote cartilage regeneration. Method In this study, we used ECM alone as a bioink in low-temperature deposition manufacturing (LDM) 3D printing and then successfully fabricated a hierarchical porous ECM scaffold incorporating GDF-5. Results Comparative in vitro experiments showed that the 7% ECM scaffolds had the best biocompatibility. After the addition of GDF-5 protein, the ECM scaffolds significantly improved bone marrow mesenchymal stem cell (BMSC) migration and chondrogenic differentiation. Most importantly, the in vivo results showed that the ECM/GDF-5 scaffold significantly enhanced in situ cartilage repair. Conclusion In conclusion, this study reports the construction of a new scaffold based on the concept of in situ regeneration, and we believe that our findings will provide a new treatment strategy for AC defect repair.

Abstract Background Hereditary transthyretin amyloidosis (hATTR) is a progressive and fatal disease with heterogenous clinical presentations, limited diagnosis and poor prognosis. This retrospective analysis study aimed to report the genotypes and phenotypes of herediary transthyretin amyloidosis (hATTR) in Chinese through a systematic review of published literature. Methods The systematic review included structured searches of peer-reviewed literature published from 2007 to 2020 of following online reference databases: PubMed, Web of Science and the literature database in China. Extracted data included sample size, personal information (sex, age, natural course, family history), mutation type, clinical milestones and reason of death. Results We described 126 Chinese patients with hereditary transthyretin amyloidosis identified through a systematic review of 30 studies. The most common genotype in the Chinese population was Gly83Arg (25, 19.8%), which most likely presented visual and neurological abnormalities without reported death. The second and third most common genotypes were Val30Met (20, 15.9%) and Val30Ala (10, 7.9%). Peripheral neurological manifestations (91, 72%) were dominant in 126 patients. The followed manifestation was autonomic neurological abnormalities (73, 58%). Half of the cases were reported to have visual disorders, and nearly one-third of the cases presented cardiac abnormalities. Among all 126 reported patients, 46.03% were classified as neurological type, 30.16% as mixed type and only 2.38% as cardiac type. In addition. Chinese patients were mostly early onset, with age of onset at 41.8 (SD: 8.9) years, and the median time from onset to death was 7.5 [IQR: 5.3] years. Patients with cardiac involvement had a shorter survival duration (log Rank (Mantel-Cox), χ2 = 26.885, P

BackgroundThe fulfillment of contractual obligations by rural-oriented tuition-waived medical students (RTMSs) to work in rural medical institutions after graduation directly impacts the improvement of rural health quality. This study aimed to not only quantitatively measure the direct impact of honesty-credit, specialty identity, and career identity on willingness to fulfill the contract of RTMSs but also to quantify the intermediary role of specialty identity and career identity between honesty-credit and willingness to fulfill the contract. The research results provided recommendations for the rural-oriented tuition-waived medical education (RTME) program to achieve its goal of training rural primary healthcare personnel.MethodsFrom March to May 2022, 1162 RTMSs were selected as the research objects. The honesty-credit, specialty identity, career identity, and willingness to fulfill the contract were quantitated using a self-completed questionnaire. Pearson's correlation analysis and structural equation modeling were used for statistical analysis and mediating effect evaluation.ResultsA total of 455 (42.3%) RTMSs had high willingness to fulfill the contract, and honesty-credit had a significant direct positive effect on willingness (β = 0.198, P < 0.001), specialty identity (β = 0.653, P < 0.001), and career identity (β = 0.180, P < 0.001). In the intermediary path between honesty-credit and willingness, career identity [95% confidence interval (CI): 0.007–0.051] had significant mediating effects. Career identity (95% CI: 0.030–0.149) also had significant mediating effects between specialty identity and willingness, and specialty identity (95% CI: 0.465–0.760) had significant mediating effects between honesty-credit and career identity. These results strongly confirmed that honesty-credit, specialty identity, and career identity are early and powerful predictors of the willingness to fulfill the contract of RTMSs.ConclusionThe honesty-credit of RTMSs can predict their willingness to fulfill the contract early, significantly and positively. For the students who fail to pass the credit assessment for many times and have a strong tendency to default, their training qualifications should be canceled in time, so that students who are truly willing to serve rural areas can enter the project, and finally achieve the policy goal of “strengthening the rural primary medical and health system”.

Introduction Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease commonly caused by pathogenic genetic variants encoding sarcomere proteins. Mavacamten, a first-in-class allosteric inhibitor of cardiac-specific myosin, has demonstrated efficacy and safety in international clinical trials of patients with symptomatic obstructive HCM (oHCM) but clinical evidence for mavacamten in the Chinese population is lacking.Methods and analysis EXPLORER-CN is a multicentre, phase III, randomised, double-blind, placebo-controlled registration trial to evaluate the efficacy and safety of mavacamten in Chinese adults with symptomatic oHCM. The study will enrol approximately 81 participants with symptomatic oHCM. Eligible participants are randomised 2:1 to receive once-daily, oral mavacamten (starting dose 2.5 mg/day), or matching placebo, for 30 weeks, followed by a long-term extension (LTE) period of 48 weeks with active treatment for all subjects. The mavacamten dose will be adjusted by pharmacokinetic (PK)/pharmacodynamic (PD) parameters during the double-blinded, placebo-controlled period and PD-only during the LTE period. The primary efficacy endpoint is change from baseline to week 30 in Valsalva left ventricular outflow tract (LVOT) peak gradient determined by Doppler echocardiography. Secondary efficacy endpoints are change in resting LVOT peak gradient, proportion of participants achieving a Valsalva LVOT peak gradient

Cardiovascular disease is a global health problem. Astragaloside IV (AS-IV) is a saponin compound extracted from the roots of the Chinese herb Astragalus. Over the past few decades, AS-IV has been shown to possess various pharmacological properties. It can protect the myocardium through antioxidative stress, anti-inflammatory effects, regulation of calcium homeostasis, improvement of myocardial energy metabolism, anti-apoptosis, anti-cardiomyocyte hypertrophy, anti-myocardial fibrosis, regulation of myocardial autophagy, and improvement of myocardial microcirculation. AS-IV exerts protective effects on blood vessels. For example, it can protect vascular endothelial cells through antioxidative stress and anti-inflammatory pathways, relax blood vessels, stabilize atherosclerotic plaques, and inhibit the proliferation and migration of vascular smooth muscle cells. Thus, the bioavailability of AS-IV is low. Toxicology indicates that AS-IV is safe, but should be used cautiously in pregnant women. In this paper, we review the mechanisms of AS-IV prevention and treatment of cardiovascular diseases in recent years to provide a reference for future research and drug development.

Abstract Backgrounds The EmPHasis-10 questionnaire is a disease-specific quality of life (QoL) measurement in patients with pulmonary hypertension. We report the results of cross-cultural validation of the Chinese version of the EmPHasis-10 and its relationship with risk stratification in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). Methods The Emphasis-10 was administered to 75 CTD-PAH patients along with the 36-item Medical Outcomes Study Short Form Survey (SF-36) and EuroQol five dimensions questionnaire (EQ-5D). The diagnosis of PAH was confirmed by right heart catheterization. Demographic and clinical data were obtained. Multivariable logistic regression was conducted based on the low risk profile assessed by a 4-strata risk assessment model (COMPERA 2.0) at follow-up. Results Date from 75 patients with CTD-PAH were analysed. The EmPHasis-10 demonstrated satisfactory reliability (Cronbach α = 0.95) and convergent validity showed by the significant relationship with WHO Functional Class (P = 0.003), SF-36 (P

Abstract. Objective:. Cardiac damage is commonly reported in patients with coronavirus disease 2019 (COVID-19) but its prevalence and impact on the long-term survival of patients remain uncertain. This study aimed to explore the prevalence of myocardial injury and assess its prognostic value in patients with COVID-19. Methods:. A single-center, retrospective cohort study was performed at the Affiliated Hospital of Jianghan University. Data from 766 patients with confirmed COVID-19 who were hospitalized from December 27, 2019 to April 25, 2020 were collected. Demographic, clinical, laboratory, electrocardiogram, treatment data and all-cause mortality during follow-up were collected and analyzed. Results:. Of the 766 patients with moderate to critically ill COVID-19, 86 (11.2%) died after a mean follow-up of 72.8 days. Myocardial injury occurred in 94 (12.3%) patients. The mortality rate was 64.9% (61/94) and 3.7% (25/672) in patients with and without myocardial injury, respectively. Cox regression showed that myocardial injury was an independent risk factor for mortality (hazard ratio: 8.76, 95% confidence interval: 4.76–16.11, P

Due to the non-renewable nature and pollution associated with fossil fuels, there is widespread research into alternative energy sources. As a novel energy device, a proton exchange membrane fuel cell (PEMFC) is considered a promising candidate for transportation due to its advantages, including zero carbon emissions, low noise, and high energy density. However, the commercialization of fuel cells faces a significant challenge related to aging and performance degradation during operation. In order to comprehensively address the issue of fuel cell aging and performance decline, this paper provides a detailed review of aging mechanisms and influencing factors from the perspectives of both the PEMFC system and the stack. On this basis, this paper offers targeted solutions to degradation issues stemming from various aging factors and presents research on aging prediction methods to proactively mitigate aging-related problems. Furthermore, to enhance prediction accuracy, this paper categorizes and analyzes the degradation index and accuracy evaluation criteria commonly employed in the existing fuel cell aging research. The results indicate that specific factors leading to aging-related failures are often addressed via targeted solving methods, corresponding to specific degradation indexes. The significance of this study lies in the following aspects: (1) investigating the aging factors in fuel cells and elucidating the multiple aging mechanisms occurring within fuel cells; (2) proposing preventive measures, solutions, and aging prediction methods tailored to address fuel cell aging issues comprehensively, thereby mitigating potential harm; and (3) summarizing the degradation index and accuracy evaluation standards for aging prediction, offering new perspectives for resolving fuel cell aging problems.

Abstract Background Rare disease patients often experience diagnosis delays or misdiagnosis, which may be due to lack of knowledge on rare diseases among physicians. Objective To assess Chinese physicians’ knowledge on specific rare diseases and identify its associated factors. Methods Thirty-four patient organizations with a unique disease of interest were invited to develop 3 knowledge questions for each rare disease to assess physicians’ knowledge on the disease that they felt most experienced in. The total knowledge score for each participant ranged from a score of 0 to 3. A national cross-sectional study conducted in a cohort of 3197 physicians from 6 provinces across western, central and eastern China. The demographic information of the participants was collected including gender, age, birthplace, income, education, hospital class, working title, working years, and specialty. A multiple linear regression analysis was performed to assess the independent associations between the physician variables and the total knowledge score. Results Two thousand, one hundred and fifteen (66.16%) of the involved physicians obtained a total knowledge score of 2 or 3. The median knowledge scores of 10 (29.4%) rare diseases were a score of 1.5 or below. Physicians with female gender (β = 0.08, p

Objective: To prepare adipose-derived stem cells (ADSCs)-embedded alginate-gelatinemicrospheres (Alg-Gel-ADSCs MSs) by electrospray and evaluate their feasibility for cartilage tissue engineering. To observe the efficacy of Alg-Gel-ADSCs MSs in repairing articular cartilage defects in SD rats. Methods: ADSCs were isolated and characterized by performing induced differentiation and flow cytometry assays. Alginate-gelatine microspheres with different gelatine concentrations were manufactured by electrospraying, and the appropriate alginate-gelatine concentration and ratio were determined by evaluating microsphere formation. Alg-Gel-ADSCs MSs were compared with Alg-ADSCs MSs through the induction of chondrogenic differentiation and culture. Their feasibility for cartilage tissue engineering was analysed by performing Live/Dead staining, cell proliferation analysis, toluidine blue staining and a glycosaminoglycan (GAG) content analysis. Alg-Gel-ADSCs MSs were implanted in the cartilage defects of SD rats, and the cartilage repair effect was evaluated at different time points. The evaluation included gross observations and histological evaluations, fluorescence imaging tracking, immunohistochemical staining, microcomputed tomography (micro-CT) and a CatWalk evaluation. Results: The isolated ADSCs showed multidirectional differentiation and were used for cartilage tissue engineering. Using 1.5 w:v% alginate and 0.5 w:v% gelatine (Type B), we successfully prepared nearly spherical microspheres. Compared with alginate microspheres, alginate gel increased the viability of ADSCs and promoted the proliferation and chondrogenesis of ADSCs. In our experiments on knee cartilage defects in SD rats in vivo, the Alg-Gel-ADSCs MSs showed superior cartilage repair in cell resides, histology evaluation, micro-CT imaging and gait analysis. Conclusions: Microspheres composed of 1.5 w:v% alginate-0.5 w:v% gelatine increase the viability of ADSCs and supported their proliferation and deposition of cartilage matrix components. ADSCs embedded in 1.5 w:v% alginate-0.5 w:v% gelatine microspheres show superior repair efficacy and prospective applications in cartilage tissue repair. The translational potential of this article: In this study, injectable adipose-derived stem cells-embedded alginate-gelatin microspheres (Alg-Gel-ADSCs MSs) were prepared by the electrospray . Compared with the traditional alginate microspheres, its support ability for ADSCs is better and shows a better repair effect. This study provides a promising strategy for cartilage tissue regeneration.

ObjectiveTo compare the disparity in framework completeness of maternal health care (MHC) system between Beijing and Shanghai and to explore the feasibility of quantitative evaluation on the degree of framework completeness of MHC system.MethodsWe systematically and extensively collected documents on MHC issued by administrative agencies of Beijing and Shanghai municipality and relevant literatures published during 2000 – 2017. Then we extracted information on the MHC system′s department, institution and organizational structure from 649 retrieved documents and literatures (232 relevant to Beijing and 417 to Shanghai) and analyzed the information quantitatively to assess framework completeness of MHC system in the two municipalities. Spearman correlation and linear regression analysis were used to analyze the relationship between framework completeness of MHC system and maternal mortality rate (MMR).ResultsThe relevant information-derived index for framework completeness of MHC system increased from 52.9% in 2000 to 78.0% in 2017 in Beijing municipality; while, the index increased from 68.5% to 85.8% in Shanghai, with a gradually increasing trend. There was an inverse correlation between the index for framework completeness of MHC system and MMR in the two municipalities.ConclusionThe framework completeness of maternal health care system was improved and the improvement facilitated maternal health care practice in Beijing and Shanghai gradually during 2000 – 2017; but some supportive departments were not embedded into the MHC system′s framework and the completeness of subsystem need to be improved. The study verified the feasibility of quantitative assessment on framework completeness of maternal health care system.

Abstract Background China has made tremendous progresses in serving the needs of its people living with rare diseases in the past decade, especially over the last 5 years. The Chinese government’s systematic approach included a series of coordinated initiatives, amongst these are: forming the Rare Disease Expert Committee (2016), funding the “Rare Diseases Cohort Study” (2016–2020), and publishing its first “Rare Disease Catalog” (2018). Herein, we present the National Rare Diseases Registry System (NRDRS)—China’s first national rare diseases registry, and the analysis of cases registered in the first 5 years ending Dec 31, 2020. Results The total 62,590 cases covered 166 disease/disease types, forming 183 disease cohorts. The data from nearly 22% of them (13,947 cases) is also linked to valuable biological samples. The average age of definitive diagnosis was 30.88 years; 36.07% of cases were under 18 years of age. Regional distribution analysis showed 60% of cases were from the more developed, wealthier East and North China, suggesting the local availability of quality care and patients’ financial status were key access factors. Finally, 82.04% of cases were registered from the five clinical departments: Neurology, Endocrine, Hematology, Cardiovascular, and Nephrology, suggesting that either these are most affected by rare diseases, or that there were disease non-specific ascertainment factors. Conclusions The preliminary analysis of the first 5-year’s data provides unique and valuable insight on rare disease distribution in China, and higlights the directions for enhancing equity, scale and utility.

Abstract Optimal transmission network reconfiguration is an important means to improve the economy and reliability of power grid. However, the traditional optimal transmission switching only considers the transmission line switching strategy and some approximate power flow models are adopted to make it tractable, such as linearisation or convex relaxation ones. In this article, first, a comprehensive network reconfiguration model considering both the line switching strategy and the bus splitting strategy inside substations is established. This optimal network reconfiguration incorporating AC power flow equations is an intractable mixed integer non‐linear programming problem. The model is first relaxed to a mixed integer second‐order cone programming to get the initial solution and an extended feasibility recovery algorithm is developed to obtain the solution satisfying the AC power flow equations. Numerical tests show that the proposed method can reduce the total generation cost and guarantee the system security while the baseline methods cannot obtain feasible solution in most cases.

Abstract Growth‐differentiation factor (GDF)‐15 is a member of transforming growth factor‐β‐related cytokine and may respond to right ventricular overload. The objective of this article was to assess the diagnosis and prognostic value of GDF‐15 in systemic lupus erythematosus‐associated pulmonary arterial hypertension (SLE‐PAH). Serum samples were obtained from 65 patients with SLE‐PAH, 51 sex and age matched patients of SLE without PAH (SLE‐non‐PAH), and 32 healthy controls. Serum GDF‐15 level was detected by enzyme‐linked immunosorbent assay and the optimal cut‐off point was determined by receiver operating characteristic curve. The primary end‐point was death from any cause and the secondary end‐point was target goal achievement (TGA). Cox regression analyses and Kaplan−Meier method were performed to identify the prognostic value of GDF‐15. Serum GDF‐15 levels were significantly higher in SLE‐PAH patients (1112.14 ± 781.80 pg/mL) than SLE‐non‐PAH patients (810 ± 408 pg/mL) and healthy controls (442 ± 139 pg/mL) at baseline. The optimal cut‐off value of GDF‐15 in the diagnosis of SLE‐PAH was 733 pg/mL (AUC = 0.84). In patients with SLE‐PAH, GDF‐15 level was associated with 6 min walking distance (ρ = −0.385, p = 0.017) and higher serum N terminal‐pro brain natriuretic peptide (NT‐proBNP) (ρ = 0.605, p 733 pg/mL were more likely to death (adjusted hazard ratio [HR] = 4.01, 95% confidence intervals [CI]: 1.23−6.27, p = 0.041) and less likely to achieve treatment goal (adjusted HR = 0.57, 95% CI: 0.23−0.79, p = 0.028). In addition, patients with simultaneous elevation of GDF‐15 and NT‐proBNP showed lower proportion of TGA (p = 0.046). In conclusion, GDF‐15 is a new and promising biomarker of development and prognosis in SLE‐PAH. The combination of GDF‐15 and NT‐proBNP may provide more accurate prognostic information.

ObjectiveTo analyze the extent of deficit in total financial investment in maternal health care (MHC), the changing trend of maternal mortality rate (MMR) and their regional disparities in Beijing and Shanghai municipality during the period from 2000 through 2017 and to explore suitable total financial investment in MHC. Methods We searched China National Knowledge Infrastructure (CNKI) and Web of Science for research literatures on financial investment in MHC in Beijing and Shanghai municipality published during 1995 – 2017. The information on deficit in total financial investment in MHC was extracted from 205 retrieved studies for calculation of a severity score to evaluate the deficit in total financial investment in MHC. Spearman correlation and linear regression analysis were used to analyze the relationship between the deficit in total financial investment in MHC and MMR. Results For Beijing municipality, the severity score for the deficit in total financial investment in MHC decreased from 3.54 in 2000 to 2.78 in 2017, while for Shanghai municipality, the score decreased from 3.94 to 1.41. Regression analysis on the correlation between the deficit severity score and MMR resulted in statistically significant regression equations for both Beijing (P < 0.05) and Shanghai (P < 0.01). Conclusion The total financial investment in maternal health care increased gradually over the years in both Beijing and Shanghai municipality during 2000 – 2017. The influence of total financial investment on maternal mortality rate is more obvious for Shanghai. The study verified the feasibility of quantitative assessment on total financial investment in maternal health care.

Left ventricular noncompaction (LVNC) is a heterogeneous cardiomyopathy that can be classified into different subtypes based on morphologic and functional features. However, the prognosis of the dilated and isolated subtypes of non-pediatric LVNC remains unknown. We retrospectively studied 101 patients with LVNC diagnosed at Peking Union Medical College Hospital from 2006 to 2022 using the Jenni criteria of transthoracic echocardiography. The patients were grouped into those with dilated LVNC (n = 64) or isolated LVNC (n = 37), and 88 patients (54 with dilated LVNC and 34 with isolated LVNC) were followed up successfully. The primary outcome was major adverse cardiovascular events (a composite of cardiovascular mortality, heart failure, severe ventricular arrhythmia, and systolic embolism). The median follow-up time was 5.24 years. The incidence of major adverse cardiovascular events was 43.2%; patients with dilated LVNC had a higher risk (adjusted hazard ratio, 4.43; 95% confidence interval, 1.24–15.81; p = 0.02) than those with isolated LVNC. None of the isolated LVNC patients had cardiovascular deaths or severe ventricular arrhythmias. The risk of systemic embolism was similar between patients with dilated and isolated LVNC. Our findings indicate that transthoracic echocardiography is a useful tool for classifying LVNC into subtypes with distinct clinical outcomes. Dilated LVNC is associated with a poor prognosis, while the isolated subtype is probably a physiological condition.

Mannan oligosaccharides (MOSs) are excellent prebiotics that are usually obtained via the enzymatic hydrolysis of mannan. In order to reduce the cost of preparing MOSs, immobilized enzymes that demonstrate good performance, require simple preparation, and are safe, inexpensive, and reusable must be developed urgently. In this study, β-mannanase was immobilized on calcium alginate (CaAlg). Under the optimal conditions of 320 U enzyme addition, 1.6% sodium alginate, 2% CaCl2, and 1 h of immobilization time, the immobilization yield reached 68.3%. The optimum temperature and pH for the immobilized β-mannanase (Man-CaAlg) were 75 °C and 6.0, respectively. The Man-CaAlg exhibited better thermal stability, a high degree of pH stability, and less substrate affinity than free β-mannanase. The Man-CaAlg could be reused eight times and retained 70.34% of its activity; additionally, the Man-CaAlg showed 58.17% activity after 30 days of storage. A total of 7.94 mg/mL of MOSs, with 4.94 mg/mL of mannobiose and 3.00 mg/mL of mannotriose, were generated in the oligosaccharide production assay. It is believed that this convenient and safe strategy has great potential in the important field of the use of immobilized β-mannanase for the production of mannan oligosaccharides.

Abstract. Percutaneous endomyocardial biopsy (EMB) is a technique that biopsy samples are obtained using bioptome through peripheral blood vessel. This technique has been used and refined more than 50 years in clinical application. Now EMB has become an important diagnostic tool in the diagnosis of myocarditis and cardiomyopathy. Department of cardiology, Peking Union Medical College Hospital (PUMCH) has performed more than 500 cases of EMB and accumulated abundant practical experience. The clinical practice guideline of percutaneous EMB in PUMCH was also established, including indications, contradictions, operation key points, sample handling and processing, post-procedure monitoring, and treatment strategy for potential complications, etc. Due to the limitation of EMB, cardiologists should collaborate with experts in echocardiography, cardiac magnetic resonance and cardiac pathology to avoid missed diagnosis and misdiagnosis. Cardiologists should pay more attention to the value of EMB and actively perform EMB to improve the diagnosis level for myocarditis and cardiomyopathy in China.

Articular cartilage defect repair is a problem that has long plagued clinicians. Although mesenchymal stem cells (MSCs) have the potential to regenerate articular cartilage, they also have many limitations. Recent studies have found that MSC-derived exosomes (MSC-Exos) play an important role in tissue regeneration. The purpose of this study was to verify whether MSC-Exos can enhance the reparative effect of the acellular cartilage extracellular matrix (ACECM) scaffold and to explore the underlying mechanism. The results of in vitro experiments show that human umbilical cord Wharton's jelly MSC-Exos (hWJMSC-Exos) can promote the migration and proliferation of bone marrow-derived MSCs (BMSCs) and the proliferation of chondrocytes. We also found that hWJMSC-Exos can promote the polarization of macrophages toward the M2 phenotype. The results of a rabbit knee osteochondral defect repair model confirmed that hWJMSC-Exos can enhance the effect of the ACECM scaffold and promote osteochondral regeneration. We demonstrated that hWJMSC-Exos can regulate the microenvironment of the articular cavity using a rat knee joint osteochondral defect model. This effect was mainly manifested in promoting the polarization of macrophages toward the M2 phenotype and inhibiting the inflammatory response, which may be a promoting factor for osteochondral regeneration. In addition, microRNA (miRNA) sequencing confirmed that hWJMSC-Exos contain many miRNAs that can promote the regeneration of hyaline cartilage. We further clarified the role of hWJMSC-Exos in osteochondral regeneration through target gene prediction and pathway enrichment analysis. In summary, this study confirms that hWJMSC-Exos can enhance the effect of the ACECM scaffold and promote osteochondral regeneration.

BackgroundIn China, COVID-19 has undoubtedly posed a huge challenge to the capacity of rural public health services. Village public health service providers are responsible for reporting and dealing with infectious diseases and public health emergencies. However, the turnover of village public health service providers is gravely threatening the stability of rural primary health system step by step. This study systematically evaluated the effects of professional identity, job satisfaction, burnout on turnover intention of village public health service providers, and further measured the mediating effect of job satisfaction and burnout between professional identity and turnover intention.MethodsFrom May to June 2019, 1,244 village public health service providers in Shandong Province were selected as the research objects. Sociodemographic characteristics, professional identity, job satisfaction, burnout and turnover intention were quantitated by self-completed questionnaire and measured by Likert 5–7 scale. Person correlation analysis, One-way ANOVA, and Structural Equation Modeling (SEM) were used for statistical analysis and mediating effect evaluation.ResultsFive hundred and sixty-four (45.3%) village public health providers had high turnover intension. Professional identity had a direct positive effect on job satisfaction (β = 0.146, p < 0.001), job satisfaction had a direct negative effect on burnout (β = 0.263, p < 0.001), and turnover intension (β = −0.453, p < 0.001), while burnout had a direct positive effect on turnover intension (β = 0.242, p < 0.001). Between professional identity and turnover intention, job satisfaction 95%CI: (−0.289)–(−0.11) had significant mediating effects. Job satisfaction 95%CI: (−0.216)–(−0.077) also had significant mediating effects between professional identity and burnout, and burnout had significant mediating effects between job satisfaction and turnover intension, 95%CI: (−0.116)–(−0.052). These results strongly confirm that professional identity, job satisfaction, and burnout are early and powerful predicators of turnover intention.ConclusionAccording to the results, medical administration and management departments should pay attention to improve the professional attraction of public health services by improving the public's understanding of the profession, reducing work intensity, timely granting of subsidy funds, improving old-age security, ensuring the income level, increasing the new force and so on, so as to reduce turnover intension and ensure the health equity of village residents.

Objective This study aimed to investigate the clinical characteristics and outcomes of pregnancy complicated by SLE-associated pulmonary arterial hypertension (SLE-PAH) in a case series and literature review.Methods This single-centre retrospective study included 10 consecutive pregnancies complicated by SLE-PAH confirmed by right heart catheterisation (RHC) at Peking Union Medical College Hospital between 2009 and 2020. A literature search was conducted and 14 pregnancy cases complicated by SLE-PAH were reviewed.Results At the time of 10 patients’ initial visits, the average age was 30.00±5.72 years and the median disease duration of SLE and PAH was 34.5 (range 1–164) months and 2 (1–51) months. Two patients carried planned pregnancy, seven patients developed PAH during pregnancy and one pregnancy was unplanned. Further, nine patients had low disease activity, with Systemic Lupus Erythematosus Disease Activity Index between 0 and 4, and 30%, 30% and 40% of patients were of WHO functional class II, III and IV, respectively. All patients were evaluated by RHC and echocardiography. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels were elevated in 70% of patients, with a median level of 776 (56–18 023) pg/mL. The median time of completed pregnancies in all patients was 31 (15–38) weeks and six patients delivered live infants. SLE activity and PAH severity improved in 70% of patients within 6 months after delivery. One patient died on the 15th day after induction of labour. In the remaining patients, all achieved a lupus low disease activity state; according to the European Society of Cardiology/European Respiratory Society risk stratification, seven were categorised at a lower risk state compared with their risk stratification during pregnancy, and two remained at intermediate risk. Additionally, 80% of patients exhibited mild impairments with WHO functional class I or II. The median NT-proBNP level was 184 (32–4003) pg/mL within 6 months after delivery. In the reviewed literature, the average age of patients was 30.09±5.37 years. The median time of completed pregnancies was 36 (28–40) weeks. More cases were planned and successful, and the survival rates of mothers and neonates were 85.71% and 92.86%, respectively.Conclusions Successful pregnancy could be possible in women with SLE-PAH if SLE-PAH treatment goals are achieved under proper therapies, careful monitoring and thorough evaluations.

BackgroundFamilial hypercholesterolemia (FH) is an autosomal-dominant genetic disorder with a high risk of premature arteriosclerotic cardiovascular disease (ASCVD). There are many alternative risk assessment tools, for example, DLCN, although their sensitivity and specificity vary among specific populations. We aimed to assess the risk discovery performance of a hybrid model consisting of existing FH risk assessment tools and machine learning (ML) methods, based on the Chinese patients with ASCVD.Materials and MethodsIn total, 5,597 primary patients with ASCVD were assessed for FH risk using 11 tools. The three best performing tools were hybridized through a voting strategy. ML models were set according to hybrid results to create a hybrid FH risk assessment tool (HFHRAT). PDP and ICE were adopted to interpret black box features.ResultsAfter hybridizing the mDLCN, Taiwan criteria, and DLCN, the HFHRAT was taken as a stacking ensemble method (AUC_class[94.85 ± 0.47], AUC_prob[98.66 ± 0.27]). The interpretation of HFHRAT suggests that patients aged 4 mmol/L were more likely to be at risk of developing FH.ConclusionThe HFHRAT has provided a median of the three tools, which could reduce the false-negative rate associated with existing tools and prevent the development of atherosclerosis. The hybrid tool could satisfy the need for a risk assessment tool for specific populations.

ObjectiveTo compare the disparity in the completeness of maternal health care (MHC) system between Beijing and Shanghai and to explore the feasibility of quantitative evaluation on the degree of the completeness.MethodsWe systematically and extensively collected documents and materials on MHC issued by administrative agencies of Beijing and Shanghai municipality published during 2000 – 2017. Then we extracted relevant information from 649 retrieved documents (232 relevant to Beijing and 417 to Shanghai) and analyzed the information quantitatively to assess the completeness of MHC system in the two municipalities. Spearman correlation and linear regression analysis were used to analyze the relationship between the completeness of MHC system and maternal mortality rate (MMR).ResultsThe relevant information-derived index for the completeness of MHC system increased from 32.4% in 2000 to 50.6% in 2017 in Beijing municipality; while, the index increased from 41.1% to 64.6% in Shanghai, respectively. For the two municipal MHC systems, the framework completeness was at a high level but the coordination and responsibility assignment among various functional institutions of the system were at a low level. There was an inverse correlation between the index for the completeness of MHC system and MMR in the two municipalities.ConclusionThe completeness of maternal health care system were improved and the improvement facilitated maternal health care practice in Beijing and Shanghai gradually during 2000 – 2017; but the coordination and responsibility assignment among various functional institutions of the system should be promoted. The study verified the feasibility of quantitative assessment on the completeness of maternal health care system.

Objective: This study evaluated the prognostic value of the multivariable risk assessment for systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH). Methods: A multicenter prospective cohort of SLE-associated PAH (CSTAR-PAH cohort) diagnosed based on right heart catheterization (RHC) was established. Baseline and follow-up records were collected. Three methods of risk assessment, including (1) the number of low-risk criteria, based on World Health Organization functional class (WHO FC), 6-min walking distance (6MWD), right atrial pressure (RAP), and cardiac index (CI); (2) the three-strata stratification based on the average risk score of four variables (WHO FC, 6MWD, RAP, and CI); and (3) the four-strata stratification based on COMPARE 2.0 model were applied. A risk-assessment method using three noninvasive low-risk criteria was applied at the first follow-up visit. Survival curves between patients with different risk groups were compared by Kaplan–Meier’s estimation and log-rank test. Results: Three-hundred and ten patients were enrolled from 14 PAH centers. All methods of stratification at baseline and first follow-up significantly discriminated long-term survival. Survival rates were also significantly different based on the noninvasive risk assessment in first follow-up visit. Survival deteriorated with the escalation of risk from baseline to first follow-up. Patients with baseline serositis had a higher rate of risk improvement in their follow-up. Conclusion: The risk assessment has a significant prognostic value at both the baseline and first follow-up assessment of SLE-associated PAH. A noninvasive risk assessment can also be useful when RHC is not available during follow-up. Baseline serositis may be a predictor of good treatment response in patients with SLE-associated PAH.

BackgroundPulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis (SSc). PAH has high mortality, and risk assessment is critical for proper management. Whether the right ventricle to pulmonary artery (RV-PA) coupling accurately assesses risk status and predicts prognosis in patients with SSc-associated PAH has not been investigated.MethodsBetween March 2010 and July 2018, 60 consecutive patients with SSc-associated PAH diagnosed by right heart catheterization were enrolled prospectively, and the mean follow-up period was 52.9 ± 27.0 months. The RV-PA coupling was assessed by the ratio of tricuspid annular plane systolic excursion (TAPSE) and pulmonary artery systolic pressure (PASP) which was obtained by transthoracic echocardiography. The simplified risk stratification strategy was applied to assess the risk level of participants, and the endpoint was a composite of all-cause death and clinical worsening.ResultsThe receiver operating characteristic (ROC) curve of the ability to determine high-risk patients identified the optimal cut-off value of the TAPSE/PASP ratio as 0.194 mm/mmHg, and the ratio appeared to be a reliable indicator in the stratification of patients with high risk (area under the curve = 0.878, ROC P-value = 0.003), which showed the highest positive likelihood ratio (LR) (5.4) and the lowest negative LR (0) among a series of echocardiographic parameters. The TAPSE/PASP ratio was an independent predictive factor (HR = 0.01, 95% CI: 0.00–0.77, P = 0.037) for the composite endpoint, and patients with a TAPSE/PASP ratio >0.194 had a better overall survival for both the composite endpoint (log-rank χ2 = 5.961, P = 0.015) and all-cause mortality (log-rank χ2 = 8.004, P = 0.005) compared to the patients with a TAPSE/PASP ≤ 0.194.ConclusionRV-PA coupling assessed by the TAPSE/PASP ratio provides added value as a straightforward and non-invasive approach for predicting risk stratification of patients with SSc-associated PAH. Meanwhile, a lower TAPSE/PASP ratio identified a subgroup with worse prognosis.

Abstract Background 99mTc-PYP scintigraphy provides differential diagnosis of ATTR cardiomyopathy (ATTR-CM) from light chain cardiac amyloidosis and other myocardial disorders without biopsy. This study was aimed to assess the diagnostic feasibility and the operator reproducibility of 99mTc-PYP quantitative SPECT. Method Thirty-seven consecutive patients who underwent a 99mTc-PYP thorax planar scan followed by SPECT and CT scans to diagnose suspected ATTR-CM were enrolled. For the quantitative SPECT, phantom studies were initially performed to determine the image conversion factor (ICF) and partial volume correction (PVC) factor to recover 99mTc-PYP activity concentration in the myocardium for calculating the standardized uptake value (SUV) (unit: g/ml). SUVmax was compared among groups of ATTR-CM, AL cardiac amyloidosis, and other pathogens (others) and among categories of Perugini visual scores (grades 0–3). The intra- and inter-operator reproducibility of quantitative SPECT was verified, and the corresponded repeatability coefficient (RPC) was calculated. Results The ICF was 79,327 Bq/ml to convert count rate in pixel to 99mTc activity concentration. PVC factor as a function of the measured activity concentration ratio in the myocardium and blood-pool was [y = 1.424 × (1 − exp(− 0.759 × x)) + 0.104]. SUVmax of ATTR-CM (7.50 ± 2.68) was significantly higher than those of AL (1.96 ± 0.35) and others (2.00 ± 0.74) (all p < 0.05). SUVmax of grade 3 (8.95 ± 1.89) and grade 2 (4.71 ± 0.23) were also significantly higher than those of grade 1 (1.92 ± 0.31) and grade 0 (1.59 ± 0.39) (all p < 0.05). Correlation coefficient (R 2) of SUVmax reached 0.966 to 0.978 with only small systematic difference (intra = − 0.14; inter = − 0.23) between two repeated measurements. Intra- and inter-operator RPCs were 0.688 and 0.877. Conclusions 99mTc-PYP quantitative SPECT integrated with adjustable PVC factors is feasible to quantitatively and objectively assess the burden of cardiac amyloidosis for diagnosis of ATTR-CM.

ObjectiveTo determine the profile of Chinese medical professionals with burnout symptoms at the national level and identify the association between capability well-being and burnout.Design and SettingA cross-sectional study in a nonrandom national sample of medical staff from 6 provinces across western, central and eastern China.ParticipantsPhysicians, medical laboratory scientists, nurses, and general practitioners aged 18 years or above who submitted a completed online questionnaire from June 2019 to January 2020 successfully (N = 25,120).Main Outcome MeasuresThe prevalence of burnout symptoms was assessed by the 22-item Maslach Burnout Inventory-Human Services Survey (MBI-HSS), which consists of three domains: emotional exhaustion (EE), depersonalization (DP), and personal achievement (PA). The overall high burnout was defined as EE score ≥27 or DP score ≥10. The capability well-being was measured by the Investigating Choice Experiments Capability Measure for Adults (ICECAP-A) and the overall ICECAP-A score was calculated using the UK value set, ranging from a score of 0–1. Multivariable logistic regression analysis was used to identify the association between well-being and the overall high burnout.ResultsAmong the 25,120 participants, 60.8% of the participants reported at least one symptom of burnout, whereas 11.2% reported all three symptoms of burnout. In the adjusted model, ICECAP-A score was independently associated with high burnout (AOR = 0.018, 95% CI = 0.015–0.022). Medical staff who were males, with shorter working years, working in tertiary hospitals, and those with the specialties of psychiatry, intensive care, emergency medicine, internal medicine, oncology, and pediatrics were at higher risk of reporting burnout symptoms.ConclusionThe burnout symptoms were relatively common among Chinese medical staff and they were found to be independently associated with capability well-being in health professionals. Interventions should be enhanced on vulnerable groups to reduce burnout and promote well-being in future studies.

Abstract Background Hereditary transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized progressive cardiomyopathy with heterogenous clinical manifestations that lead to its misdiagnosis and poor prognosis. This study was performed to describe the clinical characteristics and natural history of Chinese patients to improve clinical awareness of this condition. Methods In this study, we retrospectively investigated 23 patients with a confirmed diagnosis of hereditary ATTR-CM in Peking Union Medical College hospital from From January 1, 2000 to December 31, 2018. Results In all, 16 patients (69.6%) were males, the median age at disease onset was 45 (33,55) years old. The median duration from symptom onset to diagnosis was 30 (18,46) months. Phenotypes were classified as exclusively cardiac (n = 1, 4.3%) and mixed type (n = 22, 95.6%). The common mutations were Gly47Arg (7 patients [30.4%]) and Val30Ala (3 patients [13%]). Ventricular hypertrophy was observed in 23 (100%) patients, the mean thickness of the ventricular septum was 16.1 ± 3.9 mm, the mean thickness of the left ventricular posterior wall was 15.1 ± 2.8 mm. The mean left ventricle ejection fraction (LVEF) was 57.3 ± 11.9% and only 5 patients (21.7%) had LVEF 50% have better survival. Clinicians should be more aware of the complex clinical profile of ATTR amyloidosis to avoid misdiagnosis in practice.

Abstract Background Gastrointestinal stromal tumors (GISTs), a type of mesenchymal tumor in the gastrointestinal tract, are believed to be closely associated with PDGFRA and C-KIT mutations. Schwannoma in the stomach, which is an unusual location, is a rare disorder. The simultaneous occurrence of the two diseases is rarer than metachronous occurrences, and its pathological characteristics have not been reported to date. We present a case report on a patient with simultaneous coexistence of gastric schwannoma and GISTs. Case presentation A 39-year-old female visited our hospital complaining of intermittent abdominal pain for the previous 3 months. CT revealed a 3.4 cm slight homogeneous enhancement in the lesser curvature of the stomach; the mass was nodular soft tissue, which was removed by radical surgery. Two solid tumors with different volumes were located in the stomach. Histologically and immunohistochemically different, the larger tumor consisted of spindle cells surrounded by a peripheral lymphoid cuff, and was positive for S-100. The larger tumor was therefore classified as a gastric schwannoma. The smaller tumor was composed of medium-sized round, oval cells with amphiphilic granular cytoplasm; vacuolization was also observed. The tumor cells were positive for DOG1 and sporadically positive for CD34 and CD117. Hence, the smaller tumor was diagnosed as epithelioid GISTs. Sanger sequencing revealed that the GIST tumor cells contained a deletion mutation (c.2527_2538 del12,843–846del4), which was located in exon 18 of PDGFRA. Conclusion GISTs combined with gastric schwannoma are a considerably rare subgroup of gastric tumors. Related clinical research is comparatively weak, and the mechanism remains unknown. We reviewed related articles to provide knowledge to improve the correct identification, diagnosis and management of patients with gastric cancer. All pathologists involved in the diagnosis and clinicians involved in the treatment should be aware of this new kind of disease pattern to improve their understanding of the disease.

The Pulmonary Arterial Hypertension Symptoms and Impact Questionnaire (PAH-SYMPACT) is a PAH-specific patient-reported outcome scale assessing patients’ quality of life from four aspects: cardiopulmonary symptoms, cardiovascular symptoms, physical impacts and cognitive/emotional impacts. This study aimed to validate the Chinese version of PAH-SYMPACT and explore its relationship with risk stratification in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). In addition, 75 patients with CTD-PAH confirmed by right heart catheterization were invited to complete questionnaires including PAH-SYMPACT, the 36-item Medical Outcomes Study Short Form Survey (SF-36) and EuroQol five dimensions questionnaire (EQ-5D). The demographic, clinical, laboratory and treatment data were collected. The endpoint was treatment goal achievement status in 6–12 months after completing the questionnaires, defined as an integrated outcome. Participants’ mean age was 36.4 ± 11.9 years and the mean pulmonary arterial pressure was 38.9 ± 13.67 mmHg. The reliability of the PAH-SYMPACT domains ranged from 0.83 to 0.88. Results of factor analysis basically conformed the original PAH-SYMPACT. The treatment goal achievement (TGA) status in 6–12 months was significantly associated with physical impacts scores (odds ratio: 0.180, 95% confidence interval: 0.036–0.908, P=0.038). The Chinese version of PAH-SYMPACT is a reliable measurement to evaluate quality of life in CTD-PAH patients and is also a potential predictor of patient’s condition change in routine clinical practice.

No previous studies have investigated the predictive performance of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) prognostic equation and simplified risk score calculator in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH). We aimed to validate these prediction tools in an external cohort of patients with SLE-PAH. In this study, the validation cohort consisted of patients with SLE-PAH registered in a prospective, multicenter, nationwide database between November 2006 and May2016. The follow-up of patients was censored at 1 year. Discrimination, calibration, model fit, and risk stratification of the REVEAL prognostic equation and simplified risk score calculator were validated. As a result, a total of 306 patients with SLE-PAH were included. The 1-year overall survival rate was 91.5%. The C-index of the prognostic equation was 0.736, demonstrating reasonably good discrimination, and it was greater than that for the simplified risk score calculator (0.710). The overall calibration slope was 0.83, and the Brier score was 0.079. The risk of renal insufficiency and World Health Organization Functional Class III (WHO FC III) were underestimated, and the risk assigned to a heart rate >92 bpm in the REVEAL prognostic models was not observed in our validation cohort. Both model discrimination and calibration were poor in the very high-risk group. In conclusion, the REVEAL models exhibit good discriminatory ability when predicting 1-year overall survival in patients with SLE-PAH. Findings from both models should be interpreted with caution in cases of renal insufficiency, WHO FC III, and heart rate >92 bpm.

Aims: Systemic light-chain (AL) amyloidosis is a multisystemic disorder leading to multiple organ dysfunction and mortality that is often caused by cardiac involvement. Soluble suppression of tumorigenicity 2 (sST2) is a novel biomarker identified for risk stratification of heart disease. The aim of this study was to investigate the value of circulating sST2 levels in prognosis and mortality risk assessments for the AL amyloidosis population.Methods and Results: A total of 56 patients diagnosed with AL amyloidosis were enrolled in Peking Union Medical College Hospital (PUMCH) from January 2015 to May 2018. The relationships between the clinical parameters and overall survival (OS) and risk factors for disease progression were assessed. Additionally, receiver operating characteristic (ROC) curves, Kaplan–Meier analysis, and Cox hazard models were performed to explore the predictive value of sST2 in mortality rates. We found that the median OS of all patients was 7.3 [interquartile range (IQR) 4.4, 15.9] months. The median baseline sST2 level was 12.2 (IQR 5.1, 31.1) ng/ml, and the sST2 high group had more severe patients with a higher Mayo stage. In the ROC analysis, the area under the curve (AUC) was 0.728 [95% confidence interval (CI) 0.603–0.853] for sST2 to predict the outcomes of AL amyloidosis patients, and the optimal cutoff value was 12.34 ng/ml (sensitivity 80.2%, specificity 61.1%). Moreover, in multivariate Cox proportional hazards regression analysis, sST2 acted as an independent predictor of poor functional outcome in patients with AL amyloidosis.Conclusion: In AL amyloidosis patients, sST2 was a strong and independent prognostic biomarker for all-cause mortality, providing complementary prognostic information of a novel scoring system for risk stratification.

Injury of articular cartilage can cause osteoarthritis and seriously affect the physical and mental health of patients. Unfortunately, current surgical treatment techniques that are commonly used in the clinic cannot regenerate articular cartilage. Regenerative medicine involving stem cells has entered a new stage and is considered the most promising way to regenerate articular cartilage. In terms of theories on the mechanism, it was thought that stem cell-mediated articular cartilage regeneration was achieved through the directional differentiation of stem cells into chondrocytes. However, recent evidence has shown that the stem cell secretome plays an important role in biological processes such as the immune response, inflammation regulation, and drug delivery. At the same time, the stem cell secretome can effectively mediate the process of tissue regeneration. This new theory has attributed the therapeutic effect of stem cells to their paracrine effects. The application of stem cells is not limited to exogenous stem cell transplantation. Endogenous stem cell homing and in situ regeneration strategies have received extensive attention. The application of stem cell derivatives, such as conditioned media, extracellular vesicles, and extracellular matrix, is an extension of stem cell paracrine theory. On the other hand, stem cell pretreatment strategies have also shown promising therapeutic effects. This article will systematically review the latest developments in these areas, summarize challenges in articular cartilage regeneration strategies involving stem cells, and describe prospects for future development.

Animal models play an important role in preclinical studies, especially in tissue engineering scaffolds for cartilage repair, which require large animal models to verify the safety and effectiveness for clinical use. The small ruminant models are most widely used in this field than other large animals because they are cost-effective, easy to raise, not to mention the fact that the aforementioned animal presents similar anatomical features to that of humans. This review discusses the experimental study of tissue engineering scaffolds for knee articular cartilage regeneration in small ruminant models. Firstly, the selection of these scaffold materials and the preparation process in vitro that have been already used in vivo are briefly reviewed. Moreover, the major factors influencing the rational design and the implementation as well as advantages and limitations of small ruminants are also demonstrated. As regards methodology, this paper applies principles and methods followed by most researchers in the process of experimental design and operation of this kind. By summarizing and comparing different therapeutic concepts, this paper offers suggestions aiming to increase the effectiveness of preclinical research using small ruminant models and improve the process of developing corresponding therapies.