BACKGROUND: Congenital heart disease (CHD) is a leading cause of birth defect-related mortality. However, more recent CHD mortality data for China are lacking. Additionally, limited studies have evaluated sex, rural-urban, and region-specific disparities of CHD mortality in China. METHODS: We designed a population-based study using data from the Dataset of National Mortality Surveillance in China between 2008 and 2021. We calculated age-adjusted CHD mortality using the sixth census data of China in 2010 as the standard population. We assessed the temporal trends in CHD mortality by age, sex, area, and region from 2008 to 2021 using the joinpoint regression model. RESULTS: From 2008 to 2021, 33,534 deaths were attributed to CHD. The period witnessed a two-fold decrease in the age-adjusted CHD mortality from 1.61 to 0.76 per 100,000 persons (average annual percent change [AAPC] = -5.90%). Females tended to have lower age-adjusted CHD mortality than males, but with a similar decline rate from 2008 to 2021 (females: AAPC = -6.15%; males: AAPC = -5.84%). Similar AAPC values were observed among people living in urban (AAPC = -6.64%) and rural (AAPC = -6.12%) areas. Eastern regions experienced a more pronounced decrease in the age-adjusted CHD mortality (AAPC = -7.86%) than central (AAPC = -5.83%) and western regions (AAPC = -3.71%) between 2008 and 2021. Approximately half of the deaths (46.19%) due to CHD occurred during infancy. The CHD mortality rates in 2021 were lower than those in 2008 for people aged 0-39 years, with the largest decrease observed among children aged 1-4 years (AAPC = -8.26%), followed by infants (AAPC = -7.01%). CONCLUSIONS CHD mortality in China has dramatically decreased from 2008 to 2021. The slower decrease in CHD mortality in the central and western regions than in the eastern regions suggested that public health policymakers should pay more attention to health resources and health education for central and western regions.
Humans ; Heart Defects, Congenital/mortality* ; Male ; Female ; China/epidemiology* ; Infant ; Child, Preschool ; Adult ; Child ; Adolescent ; Infant, Newborn ; Middle Aged ; Young Adult ; Aged ; Rural Population

Metachromatic leukodystrophy (MLD) is an inherited disease caused by a deficiency of the enzyme arylsulfatase A (ARSA). Lentivirus-modified autologous hematopoietic stem cell gene therapy (HSCGT) has recently been approved for clinical use in pre and early symptomatic children with MLD to increase ARSA activity. Unfortunately, this advanced therapy is not available for most patients with MLD who have progressed to more advanced symptomatic stages at diagnosis. Patients with late-onset juvenile MLD typically present with a slower neurological progression of symptoms and represent a significant burden to the economy and healthcare system, whereas those with early onset infantile MLD die within a few years of symptom onset. We conducted a pilot study to determine the safety and benefit of HSCGT in patients with postsymptomatic juvenile MLD and report preliminary results. The safety profile of HSCGT was favorable in this long-term follow-up over 9 years. The most common adverse events (AEs) within 2 months of HSCGT were related to busulfan conditioning, and all AEs resolved. No HSCGT-related AEs and no evidence of distorted hematopoietic differentiation during long-term follow-up for up to 9.6 years. Importantly, to date, patients have maintained remarkably improved ARSA activity with a stable disease state, including increased Functional Independence Measure (FIM) score and decreased magnetic resonance imaging (MRI) lesion score. This long-term follow-up pilot study suggests that HSCGT is safe and provides clinical benefit to patients with postsymptomatic juvenile MLD.
Humans ; Leukodystrophy, Metachromatic/genetics* ; Pilot Projects ; Genetic Therapy/methods* ; Hematopoietic Stem Cell Transplantation ; Male ; Follow-Up Studies ; Female ; Lentivirus/genetics* ; Child ; Child, Preschool ; Hematopoietic Stem Cells/metabolism* ; Cerebroside-Sulfatase/metabolism* ; Adolescent

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OBJECTIVE: To observe the characteristics of changes in non-invasive hemodynamic parameters in neonates with septic shock so as to provide clinical reference for diagnosis and treatment. METHODS: A observational study was conducted. The neonates with sepsis complicated with septic shock or not admitted to neonatal intensive care unit (NICU) of the First Affiliated Hospital of Shantou University Medical College were enrolled as the study subjects, who were divided into preterm infant (< 37 weeks) and full-term infant (≥ 37 weeks) according to the gestational age. Healthy full-term infants and hemodynamically stable preterm infants transferring to NICU after birth were enrolled as controls. Electronic cardiometry (EC) was used to measure hemodynamic parameters, including heart rate (HR), mean arterial pressure (MAP), stroke volume (SV), stroke volume index (SVI), cardiac output (CO), cardiac index (CI), systemic vascular resistance (SVR) and systemic vascular resistance index (SVRI), before treatment in the septic shock group, at the time of diagnosis of sepsis in the sepsis without shock group, and before the discharge from the obstetric department or on the day of transferring to NICU in the control group. RESULTS: Finally, 113 neonates with complete data and parental consent for non-invasive hemodynamic monitoring were enrolled, including 32 cases in the septic shock group, 25 cases in the sepsis without shock group and 56 cases in the control group. In the septic shock group, there were 17 cases at the compensated stage and 15 cases at the decompensated stage. There were 21 full-term infants (20 cured or improved and 1 died) and 11 premature infants (7 cured or improved and 4 died), with the mortality of 15.62% (5/32). There were 18 full-term infants and 7 premature infants in the sepsis without shock group and all cured or improved without death. The control group included 28 full-term infants and 28 premature infants transferring to NICU after birth. Non-invasive hemodynamic parameter analysis showed that SV, SVI, CO and CI of full-term infants in the septic shock group were significantly lower than those in the sepsis without shock group and control group [SV (mL): 3.52±0.99 vs. 5.79±1.32, 5.22±1.02, SVI (mL/m²): 16.80 (15.05, 19.65) vs. 27.00 (22.00, 32.00), 27.00 (23.00, 29.75), CO (L/min): 0.52±0.17 vs. 0.80±0.14, 0.72±0.12, CI (mL×s^-1×m^-2): 40.00 (36.67, 49.18) vs. 62.51 (56.34, 70.85), 60.01 (53.34, 69.68), all P < 0.05], while SVR and SVRI were significantly higher than those in the sepsis without shock group and control group [SVR (kPa×s×L^-1): 773.46±291.96 vs. 524.17±84.76, 549.38±72.36, SVRI (kPa×s×L^-1×m^-2): 149.27±51.76 vs. 108.12±12.66, 107.81±11.87, all P < 0.05]. MAP, SV, SVI, CO and CI of preterm infants in the septic shock group were significantly lower than those in the control group [MAP (mmHg, 1 mmHg ≈ 0.133 kPa): 38.55±10.48 vs. 47.46±2.85, SV (mL): 2.45 (1.36, 3.58) vs. 3.96 (3.56, 4.49), SVI (mL/m²): 17.60 (14.20, 25.00) vs. 25.50 (24.00, 29.00), CO (L/min): 0.32 (0.24, 0.63) vs. 0.56 (0.49, 0.63), CI (mL×s^-1×m^-2): 40.01 (33.34, 53.34) vs. 61.68 (56.68, 63.35), all P < 0.05], while SVR and SVRI were similar to the control group [SVR (kPa×s×L^-1): 1 082.88±689.39 vs. 656.63±118.83, SVRI (kPa×s×L^-1×m^-2): 126.00±61.50 vs. 102.37±11.68, both P > 0.05]. Further analysis showed that SV, SVI and CI of neonates at the compensation stage in the septic shock group were significantly lower than those in the control group [SV (mL): 3.60±1.29 vs. 4.73±1.15, SVI (mL/m²): 19.20±8.33 vs. 26.34±3.91, CI (mL×s^-1×m^-2): 46.51±20.34 vs. 61.01±7.67, all P < 0.05], while MAP, SVR and SVRI were significantly higher than those in the control group [MAP (mmHg): 52.06±8.61 vs. 48.54±3.21, SVR (kPa×s×L^-1): 874.95±318.70 vs. 603.01±111.49, SVRI (kPa×s×L^-1×m^-2): 165.07±54.90 vs. 105.09±11.99, all P < 0.05]; MAP, SV, SVI, CO and CI of neonates at the decompensated stage in the septic shock group were significantly lower than those in the control group [MAP (mmHg): 35.13±6.08 vs. 48.54±3.21, SV (mL): 2.89±1.17 vs. 4.73±1.15, SVI (mL/m²): 18.50±4.99 vs. 26.34±3.91, CO (L/min): 0.41±0.19 vs. 0.65±0.15, CI (mL×s^-1×m^-2): 43.34±14.17 vs. 61.01±7.67, all P < 0.05], while SVR and SVRI were similar to the control group [SVR (kPa×s×L^-1): 885.49±628.04 vs. 603.01±111.49, SVRI (kPa×s×L^-1×m^-2): 114.29±43.54 vs. 105.09±11.99, both P > 0.05]. CONCLUSIONS Full-term infant with septic shock exhibit a low cardiac output, high vascular resistance hemodynamic pattern, while preterm infant with septic shock show low cardiac output and normal vascular resistance. At the compensated stage the hemodynamic change is low output and high resistance type, while at the decompensated stage it is low output and normal resistance type. Non-invasive hemodynamic monitoring can assist in the identification of neonatal septic shock and provide basis for clinical diagnosis and treatment.
Humans ; Shock, Septic/physiopathology* ; Infant, Newborn ; Hemodynamics ; Female ; Male ; Case-Control Studies ; Infant, Premature

Objective To develop and empirically validate the County Medical and Health Community Organizational Resilience Evaluation Scale to verify its reliability and validity,and to provide a reference for the management practice of organizational resilience in medical and health communities.Methods The health system resilience framework and the dual resilience analysis framework were used as the content framework of this scale,and the initial scale was formed by literature review reference and interviews,and two rounds of correspondence to 21 experts were conducted to form the County Medical and Health Community Organizational Resilience Evaluation Scale.In October-November 2023,the medical staff of six medical and health communities in Zhejiang Province were sampled to conduct the survey,and SPSS 25.0 software was applied to process the data.Results The internal consistency Cronbach's alpha coefficient of the scale was 0.97,and the Cronbach's alpha coefficients of the five dimensions ranged from 0.67～0.92(P＜0.001);the folded half reliability was 0.95,and the alpha coefficients of the two parts were 0.94.The results of the exploratory factor analysis showed that the KMO was 0.95,and that each dimension of the constructed Organizational Resilience Scale and the total score of the scale were positively correlated with the total score of the Strategic Flexibility Scale(P＜0.01).Conclusion The Organizational Resilience Measurement Scale designed has good reliability and reference value,and may provide a tool for assessing organizational resilience in county medical and health communities in the future.

Objective To develop and empirically validate the County Medical and Health Community Organizational Resilience Evaluation Scale to verify its reliability and validity,and to provide a reference for the management practice of organizational resilience in medical and health communities.Methods The health system resilience framework and the dual resilience analysis framework were used as the content framework of this scale,and the initial scale was formed by literature review reference and interviews,and two rounds of correspondence to 21 experts were conducted to form the County Medical and Health Community Organizational Resilience Evaluation Scale.In October-November 2023,the medical staff of six medical and health communities in Zhejiang Province were sampled to conduct the survey,and SPSS 25.0 software was applied to process the data.Results The internal consistency Cronbach's alpha coefficient of the scale was 0.97,and the Cronbach's alpha coefficients of the five dimensions ranged from 0.67～0.92(P＜0.001);the folded half reliability was 0.95,and the alpha coefficients of the two parts were 0.94.The results of the exploratory factor analysis showed that the KMO was 0.95,and that each dimension of the constructed Organizational Resilience Scale and the total score of the scale were positively correlated with the total score of the Strategic Flexibility Scale(P＜0.01).Conclusion The Organizational Resilience Measurement Scale designed has good reliability and reference value,and may provide a tool for assessing organizational resilience in county medical and health communities in the future.

The Major Principles of the Five Elements is a collection of Yin-Yang and Five Elements theories in the middle ancient period of China.The Xiangshu model of Yin-Yang and Five Elements is the basic paradigm to clarify the world and its existence."Constitution"and"Function"are a pair of important categories used in ancient philosophy to describe the relationship be-tween the nature and function of ontology.Based on the two temporal and spatial rhythms of the Heluo Heaven and Earth Five Elements view and the Jiugong Bafeng spatiotemporal view constructed by the Xiangshu model,The Major Principles of the Five Elements explains the connotation and characteristics of traditional medical constitution and function,aiming to elucidate the evolution process of the path-ogenesis of constitution and function,guide the dialectical thinking of constitution and function,and flexibly select and combine drug properties and flavors to harmonize the human constitution and function.This paper provides a unique perspective and method for ex-plaining the concept and connotation of constitution and function in traditional Chinese medicine by glimpsing the gradual changing way of thinking in traditional medicine from the Pre-Qin period to the Tang Dynasty.

BackgroundSibling relationships play a critical role in shaping anxiety symptoms in firstborn children. Anxiety symptoms often originate in early childhood and can persist into adolescence and adulthood. However， there is insufficient research on anxiety symptoms in preschool children， especially firstborn preschool children. ObjectiveTo explore the influencing factors of anxiety symptoms among firstborn preschool children， so as to provide references for the intervention of anxiety symptom for children in families with multiple children. MethodsFrom October to December 2021， a total of 8 449 children from 234 kindergartens in Longhua District of Shenzhen were included using a cluster sampling method. Sibling Inventory of Behavior （SIB） and Spence Preschool Anxiety Scale （SPAS） were used to investigate. Logistic regression analysis was used to identify influencing factors of anxiety symptoms in firstborn preschool children. ResultsA total of 8 419 （99.64%） valid questionnaires were collected. Anxiety symptoms were detected in 344（4.09%） firstborn preschool children. Statistically significant differences were observed between anxiety group and non-anxiety group in terms of household registration， monthly family income， maternal age， maternal education level， paternal education level， family living conditions and whether they are left-behind children （χ²/t=9.906， 33.490， 5.136， 13.485， 9.690， 17.332， 21.975， P<0.05 or 0.01）. Compared with non-anxiety group， children in the anxiety group scored higher on the SIB dimensions of rivalry， aggression and avoidance （t=165.322， 74.471， 286.419， P<0.01）， and lower on companionship， empathy and teaching （t=59.133， 42.417， 39.112， P<0.01）. Risk factors for anxiety symptoms in firstborn preschool children included left-behind children， as well as negative sibling relationships characterized by rivalry and avoidance （OR=1.195， 1.143， 1.260， P<0.05 or 0.01）. ConclusionFirstborn preschool children who are left-behind are more susceptible to anxiety symptoms. Negative sibling relationships， characterized by competition and avoidance， may also contribute to the emergence of anxiety symptoms in firstborn preschool children.

Objective To develop and empirically validate the County Medical and Health Community Organizational Resilience Evaluation Scale to verify its reliability and validity,and to provide a reference for the management practice of organizational resilience in medical and health communities.Methods The health system resilience framework and the dual resilience analysis framework were used as the content framework of this scale,and the initial scale was formed by literature review reference and interviews,and two rounds of correspondence to 21 experts were conducted to form the County Medical and Health Community Organizational Resilience Evaluation Scale.In October-November 2023,the medical staff of six medical and health communities in Zhejiang Province were sampled to conduct the survey,and SPSS 25.0 software was applied to process the data.Results The internal consistency Cronbach's alpha coefficient of the scale was 0.97,and the Cronbach's alpha coefficients of the five dimensions ranged from 0.67～0.92(P＜0.001);the folded half reliability was 0.95,and the alpha coefficients of the two parts were 0.94.The results of the exploratory factor analysis showed that the KMO was 0.95,and that each dimension of the constructed Organizational Resilience Scale and the total score of the scale were positively correlated with the total score of the Strategic Flexibility Scale(P＜0.01).Conclusion The Organizational Resilience Measurement Scale designed has good reliability and reference value,and may provide a tool for assessing organizational resilience in county medical and health communities in the future.

Objective To develop and empirically validate the County Medical and Health Community Organizational Resilience Evaluation Scale to verify its reliability and validity,and to provide a reference for the management practice of organizational resilience in medical and health communities.Methods The health system resilience framework and the dual resilience analysis framework were used as the content framework of this scale,and the initial scale was formed by literature review reference and interviews,and two rounds of correspondence to 21 experts were conducted to form the County Medical and Health Community Organizational Resilience Evaluation Scale.In October-November 2023,the medical staff of six medical and health communities in Zhejiang Province were sampled to conduct the survey,and SPSS 25.0 software was applied to process the data.Results The internal consistency Cronbach's alpha coefficient of the scale was 0.97,and the Cronbach's alpha coefficients of the five dimensions ranged from 0.67～0.92(P＜0.001);the folded half reliability was 0.95,and the alpha coefficients of the two parts were 0.94.The results of the exploratory factor analysis showed that the KMO was 0.95,and that each dimension of the constructed Organizational Resilience Scale and the total score of the scale were positively correlated with the total score of the Strategic Flexibility Scale(P＜0.01).Conclusion The Organizational Resilience Measurement Scale designed has good reliability and reference value,and may provide a tool for assessing organizational resilience in county medical and health communities in the future.