Impact of caregiver's eHealth literacy, financial well-being, and mental health on quality of life of pediatric patients with osteogenesis imperfecta.

OBJECTIVE: This study assesses the association between health-related quality of life (HRQoL) for pediatric patients with osteogenesis imperfecta (OI) and their caregivers' eHealth literacy (eHL), financial well-being, and mental health along with the impact of eHealth literacy on the financial well-being and mental health of OI caregivers. METHODS: Participants were recruited from a member pool of two OI patient organizations in China. Information about patients' HRQoL and their caregivers' eHL, financial well-being, and mental health was collected. Structure equation modeling (SEM) was used to estimate the relationship between the measures. The robust weighted least square mean and variance adjusted estimator was used. Three criteria, the comparative fit index, the Tucker-Lewis index, and the root mean square error of approximation, were used to evaluate the goodness-of-fit of the model. RESULTS: A total of 166 caregivers completed the questionnaires. Around 28.3% indicated that pediatric OI patients experienced problems related to mobility, and 25.3% reported difficulty doing usual activities. Around 52.4% of caregivers reported that their care receivers have some emotional problems while 8.4% reported that their care receivers have "a lot of" emotional problems. 'Some problems' on all dimensions on EQ-5D-Y was the most frequently reported health state (13.9%), and around 10.0% have no problems on all dimensions on EQ-5D-Y. Caregivers tended to show a significantly high eHL, financial well-being, and mental health when their care receivers reported no problems with usual activities and emotions. The SEM demonstrated a significant and positive relationship between eHL, financial well-being, and mental health. CONCLUSION: OI caregivers with high eHL reported satisfactory financial well-being and mental health; their care receivers rarely reported living with poor HRQoL. Providing multicomponent and easy-to-learn training to improve caregivers' eHL should be highly encouraged.

Investigating the psychometric properties of the EQ-5D-Y-3L, EQ-5D-Y-5L, CHU-9D, and PedsQL in children and adolescents with osteogenesis imperfecta.

The objective of this study was to evaluate and compare the psychometric properties of the EQ-5D-Y-3L, EQ-5D-Y-5L, CHU-9D, and PedsQL, in a sample of children and adolescents with osteogenesis imperfecta (OI). A web-based cross-sectional survey was conducted among Chinese children and adolescents with OI in 2021. The EQ-5D-Y-3L, EQ-5D-Y-5L, CHU-9D, and PedsQL were used to assess the health-related quality of life for the participants. Construct validity, including convergent and divergent validity, known-group validity, and test-retest reliability, was examined to assess the psychometric properties of the measures. A total of 157 pediatric OI patients self-completed the questionnaire. Few of them reported the full health status. A strong ceiling effect was observed for all dimensions on the EQ-5D-Y and most on CHU-9D. Most dimensions of the EQ-5D-Y and CHU-9D showed statistically significant correlations with the hypothesized PedsQL subscales. The test-retest reliability for the EQ-5D-Y-3L, EQ-5D-Y-5L, and CHU-9D was acceptable. The EQ-5D-Y-5L showed a better known-group validity than EQ-5D-Y-3L, CHU-9D, and PedsQL in differentiating patients in risk groups. CONCLUSION: The results confirmed that the EQ-5D-Y and CHU-9D are reliable and valid in pediatric OI patients. The EQ-5D-Y-5L performed better than EQ-5D-Y-3L regarding acceptability, convergent validity, and discriminatory power. WHAT IS KNOWN: • Performance of the preference-based measures has never been reported in patients with Osteogenesis imperfecta. WHAT IS NEW: • The EQ-5D-Y demonstrated higher sensitivity and discriminatory power than the CHU-9D in patients with osteogenesis Imperfecta • The EQ-5D-Y-3L performed slightly better than EQ-5D-Y-5L regarding convergent validity and discriminant ability.

SPI1-mediated autophagy of peripheral blood monocyte cells as a mechanism for sepsis based on single-cell RNA sequencing.

Autophagy has been documented to participate in immune responses and inflammatory diseases, but the mechanistic actions of monocyte autophagy in sepsis remain largely unknown. This study intends to analyze the mechanism of autophagy of peripheral blood monocyte cells (PBMCs) in sepsis based on single-cell RNA sequencing (scRNA-seq). The scRNA-seq data of PBMC samples from sepsis patients were downloaded from the GEO database, followed by identification of cell marker genes, key pathways and key genes. The bioinformatics analysis showed that the PBMC samples of sepsis patients mainly contained 9 immune cell types, among which three types of monocytes showed significant changes in cell numbers in sepsis patients. Of note, the highest autophagy score was found in the intermediate monocytes. The Annexin signaling pathway was a key pathway for the communication between monocytes and other cells. More importantly, SPI1 was predicted as a key gene in the autophagy phenotype of intermediate monocytes, and SPI1 might suppress ANXA1 transcription. The high expression of SPI1 in sepsis was confirmed by RT-qPCR and Western blot analysis. Dual luciferase reporter gene assay verified that SPI1 could bind to the promoter region of ANXA1. Furthermore, it was found that SPI1 might affect monocyte autophagy in the mouse model of sepsis through regulation of ANXA1. In conclusion, we provide insight into the mechanism underlying the septic potential of SPI1, which enhances monocyte autophagy by inhibiting the transcription of ANXA1 in sepsis.