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Background: Kenny–Caffey syndrome type 2 (KCS2) is an extremely rare inherited disorder characterized by proportionate short stature, skeletal defects, ocular and dental abnormalities, and transient hypocalcemia. It is caused by variants in FAM111A gene. Diagnosis of KCS2 can be challenging because of its similarities to other syndromes, the absence of clear hallmarks and the deficient number of genetically confirmed cases. Here, we aimed to further delineate and summarize the genotype and phenotype of KCS2, in order to get a better understanding of this rare disorder, and promote early diagnosis and intervention. Methods: We present clinical and genetic characteristics of eight newly affected individuals with KCS2 from six families, including one family with three individuals found to be a father‐to‐daughter transmission, adding to the limited literature. Furthermore, we performed a review of genetically confirmed KCS2 cases in PubMed, MEDLINE and CNKI databases. Results: There were six females and two males in our cohort. All the patients presented with short stature (100.0%). Clinical manifestations included ocular defects such as hypermetropia (5/8), dental problems such as defective dentition (3/8) and dental caries (3/8), skeletal and brain anomalies such as delayed closure of anterior fontanelle (6/8), cerebral calcification (3/8), cortical thickening (3/8) and medullary stenosis (4/8) of tubular bones. Endocrinologic abnormalities included hypoparathyroidism (5/8) and hypocalcemia (3/8). One male patient had micropenis and microorchidism. All cases harboured missense variants of FAM111A, and nucleotides c.1706 arose as a mutational hotspot, with seven individuals harbouring a c.1706G>A (p.Arg569His) variant, and one child harbouring a c.1531T>C (p.Tyr511His) variant. Literature review yielded a total of 46 patients from 20 papers. Data analysis showed that short stature, hypoparathyroidism and hypocalcemia, ocular and dental defects, skeletal features including cortical thickening and medullary stenosis of tubular bones, and seizures/spasms were present in more than 70% of the reported KCS2 cases. Conclusion: We provide detailed characteristics of the largest KCS2 group in China and present the first genetically confirmed instance of father‐to‐daughter transmission of KCS2. Our study confirms that Arg569His is the hot spot variant and summarizes the typical phenotypes of KCS2, which would help early diagnosis and intervention. [ABSTRACT FROM AUTHOR]

An in-depth understanding of the road-deposited sediments (RDS) wash-off process is essential to estimation of urban surface runoff pollution load and to designing methods to minimize the adverse impacts on the receiving waters. There are two debatable RDS wash-off views: source limited and transport limited. The RDS build-up and wash-off process was characterized to explore what determines the wash-off process to be source limited or transport limited based on twelve RDS sampling activities on an urban road in Beijing. The results showed that two natural rain events (2.0 mm and 23.2 mm) reduced the total RDS mass by 30%–40%, and that finer particles (< 105 μm) contributed 60%–80% of the wash-off load. Both single- and multi-rain events caused the RDS particle grain size to become coarser, while dry days made the RDS particle grain size finer. These findings indicated that the bulk RDS particles wash-off tends to be transport limited, but that finer particles tend to be source limited. To further explore and confirm the results of the field experiment, a total of 40 simulated rain events were designed to observe the RDS wash-off with different particle size fractions. The finer particles have a higher wash-off percentage ( F w ) than the coarser particles, and the F w values provide a good view to characterize the wash-off process. The key conclusions drawn from the combined field and simulated experiments data are: (i) Finer and coarser particle wash-off processes tend to be source limited and transport limited, respectively. (ii) The source and transport limited processes occur during the initial period (the first flush) and later periods, respectively. (iii) The smaller and larger rain events tend to be transport limited and source limited, respectively. Overall, the wash-off process is generally a combination of source and transport limited processes. [ABSTRACT FROM AUTHOR]

This investigation into the Little Ice Age (LIA) climate discrepancies explores sea surface temperature (SST) anomalies in the South China Sea (SCS) using monthly resolved coral Sr/Ca proxies. Results show that, during the LIA, while overall summer and annual SSTs were cooler compared to the Medieval Climate Anomaly and the Current Warm Period, winters were unexpectedly warmer. This warming is corroborated by another coral-based study and aligns with terrestrial data from high-latitude regions reporting similar warmer winters during the LIA. The analysis suggests that these higher winter SSTs in the SCS were likely due to a weakened East Asian winter monsoon and stronger El Niño events. The presence of warmer winters in various regions contradicts the traditional view of a consistently cold LIA, indicating complex regional climate variations influenced by atmospheric and oceanic interactions. This work underscores the need to reassess our understanding of the LIA's heterogeneous climate impacts globally. • LIA study finds unexpected warmer winter SSTs in the SCS via coral Sr/Ca data. • LIA's warm SSTs are possibly due to weaker winter monsoons and increased El Niño. • SCS coral records echo unexpected warmer high-latitude winters during the LIA. • The findings challenge the idea of uniformly cold LIA and suggest complex climates. [ABSTRACT FROM AUTHOR]

The South China Sea (SCS) is the major source of water vapor to China and East Asia. Modern observations, numerical simulations, and paleoclimate records suggest that on seasonal and annual timescales, variability in SCS precipitation is controlled mainly by the East Asian Monsoon and El Niño–Southern Oscillation (ENSO), respectively. Nevertheless, due to the lack of long-term observational records, our understanding of its decadal variability is limited. Because the understanding of decadal variability in SCS precipitation is fundamental in accurately predicting precipitation patterns in China and East Asia using climate models, it is necessary to obtain preinstrumental records of precipitation using climatic proxies in high resolution archives. We demonstrate the potential of coral Δδ18O, derived from Sr/Ca and δ18O measurements in the coral skeleton, as a proxy for precipitation in the SCS on different timescales. We then use this proxy to reconstruct decadal variability throughout the 20th century. The results suggest that the monsoon, Pacific Decadal Oscillation, ENSO, and Atlantic Multidecadal Oscillation are not significant forcings of decadal variability in precipitation over the SCS. In constrast, the North Atlantic Oscillation (NAO) might have modulated the number of tropical cyclones passing through the SCS and induced more (fewer) tropical cyclones and more (less) precipitation during its negative (positive) phases on decadal timescales during the past century. However, future numerical modeling studies are still required to improve our understanding of the underlying mechanism behind the relationship between the NAO and decadal variability in SCS precipitation. • Coral Δδ18O records can be used to reconstruct decadal variability in precipitation in the SCS during the 20th century. • The monsoon, PDO, ENSO, and AMO are not significant forcings of decadal variability in precipitation over the SCS. • The dominant control on decadal variability in SCS precipitation is the NAO via modulation of tropical cyclone activity. [ABSTRACT FROM AUTHOR]

Mo isotopic composition in large rivers is very important for understanding global Mo cycle. At present, temporal variation signatures in the δ 98/95 Mo in large rivers have not been investigated, which hinder a comprehensive understanding on the mechanism for the variations of Mo isotopic compositions in river water. In this study, we report a one-year-long time series (March 2010 to March 2011) of the δ 98/95 Mo of both the water and suspended particulates collected at Guiping, from the middle reaches of the Xijiang River (XJR), and of the water δ 98/95 Mo from the lower reaches of the Huanghe River (HHR, or Yellow River) collected at Lijin, China. The results indicate that the temporal variations in the concentration and δ 98/95 Mo of dissolved Mo in the XJR range from 4.32 to 10.5 nmol/L (mean 7.31 nmol/L) and 1.04‰ to 1.31‰ (relative to NIST 3134) (mean 1.20‰), respectively, but that the particulates have a lower δ 98/95 Mo (− 0.18‰ to 0.58‰). Analysis of the suspended particulates and other chemical parameters of the river water suggests that the weathering of silicates and sulfides is the main contributor to the dissolved Mo content in the XJR. Subsequently, the highly efficient preferential trapping of lighter δ 98/95 Mo by weathering products such as clay minerals, Fe–Mn oxides, and organic materials in soils and saprolites, which are abundant in the tropical catchment, is the key to the heavy Mo isotope signatures in the XJR. Furthermore, it seems that neither Mo-scavenging by suspended particulates during riverine transportation, nor the weathering of sulfates, significantly influences the δ 98/95 Mo of the XJR water. Cross comparison among the δ 98/95 Mo in the waters of the three largest rivers in China (the Changjiang (CJR), HHR, and XJR) indicates that the HHR has the highest Mo concentrations, but the lightest δ 98/95 Mo, the XJR has the lowest Mo concentrations but the heaviest δ 98/95 Mo, and the values for the CJR are intermediate between those for the other two rivers. This supports the conclusion that the efficiency of the selective trapping of Mo by soils and saprolites is the main factor controlling δ 98/95 Mo in these large rivers. The tropical/subtropical XJR catchment experiences intense chemical weathering, the semi-arid temperate HHR catchment experiences very little, and the CJR catchment falls somewhere between the two. Such a relationship between water Mo concentrations and δ 98/95 Mo agrees with previous observations from many large rivers worldwide. It is therefore suggested that chemical weathering on continents is the key to variations in the isotopic composition of Mo in the waters of large rivers. [ABSTRACT FROM AUTHOR]

Background: The social network of core members can affect the performance of the organization, while there is a lack of research on the relationship between the social network of core members of social organizations and individual performance in the field of aged care services. This study aimed to explore the relationship between social network and individual performance of core members from social organizations engaged in aged care services and explore measures to promote the development of aged care services., Methods: We used a multi-stage stratified sampling method to conduct a cross-sectional study and collected the required data in six cities in Anhui Province, China. Univariate analysis and binary logistic regression were used to estimate the relationship between social network and individual performance., Results: Our results indicated that core members with higher social network scores were more likely to yield better individual performance, including receiving awards or recognitions related to aged care services (AOR=2.534; 95% CI: 1.397-4.596). Moreover, teams led by the core members were more likely to receive awards or recognitions related to aged care services (AOR=2.930; 95% CI: 1.740-4.933). The core members or the teams led by them were more likely to be reported by the media (AOR=1.748; 95% CI: 1.030-2.966) and participate in the drafting or discussion of local aged care service standards or service specifications (AOR=2.088; 95% CI: 1.093-3.911). In addition, demographic variables such as gender, marital status, and education of core members were significantly related to their performance (P<0.05)., Conclusions: The social network of core members of aged care service social organizations has an impact on their individual performance. To improve the performance of the core members of senior citizens services and organizations, relevant measures should be taken from the government, social organizations and core members to strengthen the social network construction of core members., (© 2023. The Author(s).)

Background: Social support and anxiety have a major impact on later life quality in rural, older people in elderly caring social organizations (SOs). This study aimed to explore the relationship between social support and anxiety and their relevant influential factors among rural older people in elderly caring SOs in Anhui Province, China., Methods: This cross-sectional study was conducted through a multi-stage stratified cluster random sampling method. Independent t -tests, one-way ANOVA, Mann-Whitney U test, Kruskal-Wallis H test, and a generalized linear model were employed., Results: A significantly negative association between friends' support and anxiety were found among rural older people in elderly caring SOs. Statistically significant relationships were found between social support and gender, marital status, education level, whether visited by relatives, and institutional satisfaction. Similarly, anxiety was associated with gender, institutional satisfaction, comorbid chronic diseases, and friends' support., Conclusions: Social support from friends plays an important role in preventing and regulating anxiety among rural older people, especially those from elderly caring SOs. To reduce the occurrence and level of anxiety among rural elderly in elderly caring SOs, an effort should be given to strengthening social support, improving institutional satisfaction, and emphasizing comorbid chronic diseases.

Objective: Turnover intention of employees in elderly caring social organizations has a significant impact on elderly care service delivery. This study investigated the associated factors of turnover intention among employees of elderly caring social organizations in Anhui Province, China., Methods: A total of 605 participants were selected using a multi-stage stratified random sampling method. A self-administered questionnaire was used to collect information on socio-demographic, social support, and turnover intention from the participants. The data was analyzed through descriptive statistical analysis, one-way variance analysis, Spearman correlation analysis, and multiple linear regression were used to analyze the factors related to turnover intention., Results: Results of our study showed that the total score of turnover intention, turnover intention I (possibility of quitting a current job),turnover intention II (motivation to find other jobs) and turnover intention III (obtaining the external possibility of work) were 8.84, 2.32, 2.38, and 4.14, respectively. Social support negatively correlated with turnover intention I and turnover intention II. However, it showed positive correlation with turnover intention III and total turnover intention scores; turnover intentionI (coefficient: - 0.082), turnover intention II (coefficient: - 0.071), turnover intention III (coefficient: 0.19), Total score of turnover intention (coefficient: 0.093). Ethnic group, age, education level, and job satisfaction were associated with turnover intention., Conclusion: Improvement of social support play an important role in reducing the turnover intention of employees in elderly caring social organizations. It is important to increase organizational commitment and strengthen psychological empowerment, combined with decreasing job burnout for stability., (© 2021. The Author(s).)

This study adopted a spillover-crossover model to examine the roles of personality and perceived social support as antecedents of the work-family interface among dual-earner couples in China. Married couples (N = 306) from 2 major cities in China (Shanghai and Jinan) completed questionnaires measuring a relationship-oriented personality trait (i.e., family orientation), perceived family and work support, and work-family conflict and enhancement. The results showed that family orientation and perceived family support was positively associated with family-to-work enhancement and negatively associated with family-to-work conflict for both husbands and wives. Perceived work support was positively associated with family-to-work enhancement for wives and negatively associated with work-to-family conflict for husbands. Similarities in family orientation between partners were positively correlated with the individual's family-to-work enhancement. This study also illustrated the crossover of the work-family interface between dual-earner couples by using the actor-partner interdependence model. The pattern of associations between personality trait and perceived social support varied by gender. Husbands' family orientation was negatively correlated with work-to-family enhancement experienced by wives, and husbands' perceived work support was positively correlated with work-to-family enhancement experienced by wives. Wives' perceived work support was positively correlated with family-to-work conflict experienced by husbands.