PFDN4 as a Prognostic Marker Was Associated with Chemotherapy Resistance through CREBP1/AURKA Pathway in Triple-Negative Breast Cancer.

Breast cancer is the most common malignancy and its incidence is increasing. It is currently mainly treated by clinical chemotherapy, but chemoresistance remains poorly understood. Prefolded proteins 4 (PFDN4) are molecular chaperone complexes that bind to newly synthesized polypeptides and allow them to fold correctly to stabilize protein formation. This study aimed to investigate the role of PFDN4 in chemotherapy resistance in breast cancer. Our study found that PFDN4 was highly expressed in breast cancer compared to normal tissues and was statistically significantly associated with stage, nodal status, subclasses (luminal, HER2 positive and triple negative), triple-negative subtype and disease-specific survival by TCGA database analysis. CRISPR knockout of PFDN4 inhibited the growth of 89% of breast cancer cell lines, and the triple-negative cell line exhibited a stronger inhibitory effect than the non-triple-negative cell line. High PFDN4 expression was associated with poor overall survival in chemotherapy and resistance to doxorubicin and paclitaxel through the CREBP1/AURKA pathway in the triple-negative MDAMB231 cell line. This study provides insightful evidence for the value of PFDN4 in poor prognosis and chemotherapy resistance in breast cancer patients.

Detection of Interleukin-1 ß (IL-1ß) in Single Human Blastocyst-Conditioned Medium Using Ultrasensitive Bead-Based Digital Microfluidic Chip and Its Relationship with Embryonic Implantation Potential.

The implantation of human embryos is a complex process involving various cytokines and receptors expressed by both endometrium and embryos. However, the role of cytokines produced by a single embryo in successful implantation is largely unknown. This study aimed to investigate the role of IL-1ß expressed in a single-embryo-conditioned medium (ECM) in embryo implantation. Seventy samples of single ECM were analyzed by a specially designed magnetic-beads-based microfluidic chip from 15 women. We discovered that IL-1ß level increased as the embryo developed, and the difference was significant. In addition, receiver operator characteristic (ROC) curves analysis showed a higher chance of pregnancy when the IL-1ß level on day 5 ECM was below 79.37 pg/mL and the difference between day 5 and day 3 was below 24.90 pg/mL. Our study discovered a possible association between embryonic proteomic expression and successful implantation, which might facilitate single-embryo transfer in the future by helping clinicians identify the embryo with the greatest implantation potential.

Anatomical Anal Stenosis after PPH: Insights from a Retrospective Study and Rat Model.

High-grade hemorrhoids are usually recommended to receive operational treatments. However, these traditional surgeries are associated with severe postoperative pain. A procedure for prolapse and hemorrhoids (PPH), a circular staple device, has been developed to improve short-term outcomes, including reducing the severity of postoperative pain. PPH, compared to conventional surgery, has been associated with the incidence of anatomical anal stenosis. The causes of stenosis after PPH are not yet clear. We first analyzed the complications of our patients with PPH, and then developed a rat model to verify the tension force of PPH using Hematoxylin-eosin, Masson's trichrome, immunohistochemistry, and immunofluorescence staining. Our clinical data showed that PPH significantly improved postoperative pain, but that it resulted in higher incidences of complications, including anal stenosis, than hemorrhoidectomy. We simulated the status of PPH and developed a rat model to verify PPH's tension force, including the scarring area and the deposition of proinflammatory factors, angiogenic factors, and fibrotic factors. The tension wound histological data showed more extensive granulation tissue and inflammatory cell infiltration and a thicker epidermis than the control group on day 12 post-operation and tension treatment. In addition to IL-1ß and IL-10 cytokines on day 3 and IL-1ß, IL-6, and IL-10 cytokines on day 12 post-operation in the tension group, two angiogenic factors, CD31 and VEGF-A, were found to have a more significant expression on day 7 post-operation in the tension group. The mean scar area was larger and the distribution of fibrotic proteins (collagen 1, α-SMA, CTGF, and MMP2) in the tension group was significantly broader than in the control on day 12 post-operation and tension treatment. Based on the findings of our animal model, the development of a lesser tensile force for PPH to decrease the deposition of proinflammatory factors, angiogenic factors, and fibrotic factors is urgently required.

Sprayable hydroxypropyl chitin/collagen extract of Ampelopsis brevipedunculata hydrogel accelerates wound healing.

OBJECTIVE: Keeping a wound moist can allow effective and rapid healing, and it can control the formation of scabs, thereby allowing cell proliferation and epithelial formation. When regularly changing a dressing, thermosensitive hydrogel as a moist dressing does not cause a secondary wound from adhesion. The main aim of this study was to evaluate the effect of a new sprayable thermosensitive hydrogel on wound healing. METHOD: The hydrophobic N-acetyl group of chitin was removed by microwave reaction with lye until the degree of acetylation was 60%, followed by reaction with propylene oxide to obtain hydroxypropyl chitin (HPCH) with a degree of substitution of 40%. After mixing HPCH with fish scale collagen (FSC), a thermosensitive hydrogel with a gel temperature of 26.5°C was obtained. Ampelopsis brevipedunculata extracts (ABE), which have been found to accelerate wound repair and improve healing, were added. HPCH/FSC is not toxic to the mouse L929 cell line and forms a hydrogel at body surface temperature. It can be easily sprayed on a wound. The HPCH/FSC has a three-dimensional network porous structure with a swelling ratio of 10.95:1 and a water vapour transmission rate of 2386.03±228.87g/m2/day; it can facilitate the penetration of water and air, and promote absorption of wound exudate. Wound repair was performed on five Sprague-Dawley rats. Each rat had three wounds, which were treated with medical gauze, HPCH/FSC and HPCH/FSC/ABE, respectively. RESULTS: The wounds in the HPCH/FSC/ABE group recovered the fastest in vivo, the mature wound site was smoother, the re-epithelialisation was even and thicker, and the angiogenesis developed rapidly to the mature stage. CONCLUSION: In this study, HPCH/FSC/ABE thermosensitive hydrogel was shown to effectively accelerate wound healing and was convenient for practical application.

[Characteristics of Vertical Distribution and Environmental Factors of Antibiotics in Quaternary Sedimentary Column in Urban Areas].

Antibiotics easily remain in sediments after migrating from the surface to the subsurface due to water-rock interactions, posing a risk of secondary release to groundwater. To investigate the vertical distribution characteristics and environmental impact factors of antibiotics, five 30 m quaternary sediment columns were drilled and stratified near the hospital, and five major classes of antibiotics and sulfonamide metabolites were tested and analyzed. The results showed that:① the antibiotic content in the sediments ranged from 3.05 to 107.03 µg·kg-1, and all of the target antibiotics were detected except lomefloxacin, of which ofloxacin and oxytetracycline were the most important antibiotics in the study area. ② The antibiotics did not show a strict downward trend in the vertical direction but varied with the lithological stratification. ③ Antibiotics were primarily deposited in the clay layer and varied with the fluctuation of the groundwater level. ④ The results of redundancy analysis between antibiotics and environmental factors suggested that pH and TOC controlled the fate and transformation of antibiotics through influencing the adsorption of antibiotics by sediments. The risk of antibiotic contamination from hospital wastewater seepage into the subsurface environment should be taken seriously.

Macrophages enhance regeneration of lateral line neuromast derived from interneuromast cells through TGF-ß in zebrafish.

Macrophages play a pivotal role in the response to injury, contributing significantly to the repair and regrowth of damaged tissues. The external lateral line system in aquatic organisms offers a practical model for studying regeneration, featuring interneuromast cells connecting sensory neuromasts. Under normal conditions, these cells remain dormant, but their transformation into neuromasts occurs when overcoming inhibitory signals from Schwann cells and posterior lateral line nerves. The mechanism enabling interneuromast cells to evade inhibition by Schwann cells remains unclear. Previous observations suggest that macrophages physically interact with neuromasts, nerves, and Schwann cells during regeneration. This interaction leads to the regeneration of neuromasts in a subset of zebrafish with ablated neuromasts. To explore whether macrophages achieve this effect through secreted cytokines, we conducted experiments involving tail amputation in zebrafish larvae and tested the impact of cytokine inhibitors on neuromast regeneration. Most injured larvae remarkably regenerated a neuromast within 4 days post-amputation. Intriguingly, removal of macrophages and inhibition of the anti-inflammatory cytokine transforming growth factor-beta (TGF-ß) significantly delayed neuromast regeneration. Conversely, inhibition of the pro-inflammatory cytokines interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α) had minor effects on the regeneration process. This study provides insights into how macrophages activate interneuromast cells, elucidating the pathways underlying neuromast regeneration.

Aberrant trophoblastic differentiation in human cancer: An emerging novel therapeutic target (Review).

Various types of human cancer may develop aberrant trophoblastic differentiation, including histological changes and altered expression of ß­human chorionic gonadotropin (ß­hCG). Aberrant trophoblastic differentiation in epithelial cancer is usually associated with poor differentiation, tumor metastasis, unfavorable prognosis and treatment resistance. Since ß­hCG­targeting vaccines have failed in an early phase II trial, it is crucial to obtain a better understanding of the molecular pathogenesis of trophoblastic differentiation in human cancer. The present review summarizes the clinical and translational research on this topic with the aim of accelerating the development of an effective targeted therapy. Ectopic expression of ß­hCG promotes proliferation, migration, invasion, vasculogenesis and epithelial­mesenchymal transition (EMT) in vitro, and enhances metastatic and tumorigenic capabilities in vivo. Signaling cascades modulated by ß­hCG include the TGF­ß receptor pathway, EMT­related pathways, the c­MET receptor tyrosine kinase and mitogen­activated protein kinase/ERK pathways, and the SMAD2/4 pathway. Taken together, these findings indicated that TGF­ß receptors, c­MET and ERK1/2 are potential therapeutic targets. Nevertheless, further investigation on the molecular basis of aberrant trophoblastic differentiation is mandatory to improve the design of precision therapy for this aggressive type of human cancer.

Cold stress induces differential gene expression of retained homeologs in Camelina sativa cv Suneson.

Camelina sativa (L.) Crantz, a member of the Brassicaceae, has potential as a biofuel feedstock which is attributable to the production of fatty acids in its seeds, its fast growth cycle, and low input requirements. While a genome assembly is available for camelina, it was generated from short sequence reads and is thus highly fragmented in nature. Using long read sequences, we generated a chromosome-scale, highly contiguous genome assembly (644,491,969 bp) for the spring biotype cultivar 'Suneson' with an N50 contig length of 12,031,512 bp and a scaffold N50 length of 32,184,682 bp. Annotation of protein-coding genes revealed 91,877 genes that encode 133,355 gene models. We identified a total of 4,467 genes that were significantly up-regulated under cold stress which were enriched in gene ontology terms associated with "response to cold" and "response to abiotic stress". Coexpression analyses revealed multiple coexpression modules that were enriched in genes differentially expressed following cold stress that had putative functions involved in stress adaptation, specifically within the plastid. With access to a highly contiguous genome assembly, comparative analyses with Arabidopsis thaliana revealed 23,625 A. thaliana genes syntenic with 45,453 Suneson genes. Of these, 24,960 Suneson genes were syntenic to 8,320 A. thaliana genes reflecting a 3 camelina homeolog to 1 Arabidopsis gene relationship and retention of all three homeologs. Some of the retained triplicated homeologs showed conserved gene expression patterns under control and cold-stressed conditions whereas other triplicated homeologs displayed diverged expression patterns revealing sub- and neo-functionalization of the homeologs at the transcription level. Access to the chromosome-scale assembly of Suneson will enable both basic and applied research efforts in the improvement of camelina as a sustainable biofuel feedstock.

Gut Microbiome-Estrobolome Profile in Reproductive-Age Women with Endometriosis.

Microbiota is associated with our bodily functions and microenvironment. A healthy, balanced gut microbiome not only helps maintain mucosal integrity, prevents translocation of bacterial content, and contributes to immune status, but also associates with estrogen metabolism. Gut dysbiosis and estrobolome dysfunction have hence been linked to certain estrogen-dependent diseases, including endometriosis. While prior studies on microbiomes and endometriosis have shown conflicting results, most of the observed microbial differences are seen in the genital tract. This case-control study of reproductive-age women utilizes their fecal and urine samples for enzymatic, microbial, and metabolic studies to explore if patients with endometriosis have distinguishable gut microbiota or altered estrogen metabolism. While gut ß-glucuronidase activities, microbial diversity, and abundance did not vary significantly between patients with or without endometriosis, fecal samples of patients with endometriosis were more enriched by the Erysipelotrichia class and had higher folds of four estrogen/estrogen metabolites. Further studies are needed to elucidate what these results imply and whether there indeed is an association or causation between gut microbiota and endometriosis.

The experience of eating for older nursing home residents with dysphagia: A qualitative descriptive study.

BACKGROUND: Understanding the experience of eating for nursing home residents with dysphagia is essential for developing effective and holistic compensatory intervention programmes for older adults with dysphagia. However, there is a lack of studies on the experience of eating for older adults with dysphagia, especially for individuals in Asian cultures. OBJECTIVES: This study aimed to understand the experience of eating for older nursing home residents who have difficulty swallowing (dysphagia), which is often a problem for this population. METHODS: This qualitative descriptive study recruited older nursing home residents with dysphagia from facilities in central Taiwan. Residents were recruited by purposive sampling. Data were collected through individual in-depth semistructured face-to-face interviews. Interviews were audio-recorded and transcribed interview data were analysed with content analysis. RESULTS: A total of 20 residents participated in the study; the mean age was 78.7 years (standard deviation = 8.54 years); male and female residents were equally represented. The main core theme describing the experience of eating for nursing home residents with dysphagia was irregular coughing, which often occurred spontaneously. Three subthemes described how residents responded: making adjustments to eating and swallowing, receiving assistance from NH staff and fear of eating. CONCLUSIONS: Our findings can serve as an evidence-based reference for clinical care aimed at nursing home residents with dysphagia. Support programmes that provide safe swallowing skills and emotional support for managing dysphagia are recommended. IMPLICATIONS FOR PRACTICE: Nursing home residents with dysphagia should receive interventions focussed on self-supporting care, training in swallowing skills and emotional support.

A Comparative Study about the Neuroprotective Effects of DHA-Enriched Phosphatidylserine and EPA-Enriched Phosphatidylserine against Oxidative Damage in Primary Hippocampal Neurons.

Nerve damage caused by accumulated oxidative stress is one of the characteristics and main mechanisms of Alzheimer's disease (AD). Previous studies have shown that phosphatidylserine (PS) rich in eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) plays a significant role in preventing and mitigating the progression of AD. However, whether DHA-PS and EPA-PS can directly protect primary hippocampal neurons against oxidative damage has not been studied. Here, the neuroprotective functions of DHA-PS and EPA-PS against H2O2/t-BHP-induced oxidative damage and the possible mechanisms were evaluated in primary hippocampal neurons. It was found that DHA-PS and EPA-PS could significantly improve cell morphology and promote the restoration of neural network structure. Further studies showed that both of them significantly alleviated oxidative stress-mediated mitochondrial dysfunction. EPA-PS significantly inhibited the phosphorylation of ERK, thus playing an anti-apoptotic role, and EPA-PS significantly increased the protein expressions of p-TrkB and p-CREB, thus playing a neuroprotective role. In addition, EPA-PS, rather than DHA-PS could enhance synaptic plasticity by increasing the expression of SYN, and both could significantly reduce the expression levels of p-GSK3ß and p-Tau. These results provide a scientific basis for the use of DHA/EPA-enriched phospholipids in the treatment of neurodegenerative diseases, and also provide a reference for the development of related functional foods.

Longitudinal analysis of the impact of smoking exposure on atopic indices and allergies in early childhood.

Background: Exposure to smoking is recognized as a health hazard; however, a longitudinal analysis of the impact of smoking exposure in families on the allergic reactions related to childhood atopic diseases has not been well addressed. Methods: Children who completed a three-year follow-up period from the birth cohort were included in this study. The history of smoking exposure was recorded, and the urine cotinine levels were measured at 1 and 6 months, and 1, 2, and 3 years of age. Specific IgE levels against food and mite allergens were measured at age 6 months, and 1, 2, and 3 years. Their relevance to family smoking exposure and the subsequent development of atopic diseases was also analyzed. This study was approved by the Ethics Committee of Chang Gung Memorial Hospital (No. 102-1842C). Results: A total of 198 infants were enrolled in this study. The prevalence of passive smoking exposure among these children was as high as 45%. The urine cotinine levels were significantly higher in children with history of smoking exposure (P < 0.001). At 6 months of age, the food-specific IgE levels and the prevalence of eczema were significantly higher in children with smoking exposure than in those without smoking exposure (P < 0.05). By contrast, the urine cotinine levels were significantly higher in children with IgE sensitization (>100 kU/L, P < 0.05) at 3 years of age, which was also significantly associated with a higher prevalence of allergic rhinitis and development of asthma (P < 0.01). Conclusion: Family smoking exposure appears to be strongly associated with food sensitization in infancy and with IgE production in later childhood. This could potentially increase the susceptibility of developing infantile eczema and subsequent childhood airway allergies.

Association of Body Mass Index and Cardiometabolic Factors With Elderly Suicide: A Cohort Study of 101,518 Older Taiwanese.

BACKGROUND: Older people have the highest suicide rate across age groups in most countries. The prevalence of cardiometabolic risk factors also increases with age. We investigated the association between body mass index (BMI), cardiometabolic risk factors, and suicide in a large cohort of older people in Taiwan. METHODS: We conducted a cohort study using data from an elderly health examination program in Taipei City, Taiwan (2005-2010), linked to the national cause-of-death data files. We used competing risk Cox regression models to investigate the associations of BMI (kg/m2) and cardiometabolic factors with suicide after adjusting for sex, age, socioeconomic variables, chronic diseases, psychological distress, and cognitive function. RESULTS: Among 101,518 individuals aged ≥ 65 years, 92 died by suicide during an average follow-up of 3.9 years. Underweight (BMI<18.5) was associated with increased suicide risk (adjusted hazard ratio [aHR]=2.33, 95% confidence interval [CI] 1.20-4.52) (reference: normal weight). Low diastolic blood pressure was associated with increased suicide risk - aHR was 0.51 (95% CI 0.29-0.91) and 0.55 (95% CI 0.31-0.99) for the third and fourth quartiles of diastolic blood pressure (reference: the lowest quartile), respectively. Older people with a higher waist circumference (aHR per 1-standard-deviation increase=0.60 [95% CI 0.37-0.98]) and a higher number of metabolic syndrome criteria (aHR per 1-criterion increase=0.65 [95% 0.46-0.92]) had lower suicide risk. Systolic blood pressure, pulse rate, fasting blood glucose, and lipid profiles were not associated with suicide risk. CONCLUSIONS: Underweight, low diastolic blood pressure, and low waist circumference may be markers of increased suicide risk in older people.

Dual role of sprouty2 as an inhibitor of RAS/ERK-driven proliferation and a promoter of cancer invasion in KRAS wild-type colorectal cancer.

Sprouty2 (SPRY2) is known to inhibit the RAS/MAPK/ERK pathway, and is a potential study target for cancer. The effect of SPRY2 in colorectal cancer (CRC) and whether it is influenced by KRAS mutation are not known. We manipulated SPRY2 gene expression and used an activating KRAS-mutant plasmid to determine its effect on CRC cell function in vitro and/or in vivo. We performed SPRY2 immunohistochemical staining in 143 CRC specimens and analyzed the staining results with various clinicopathological characteristics in relation to KRAS mutation status. SPRY2 knockdown in Caco-2 cells carrying the wild-type (WT) KRAS gene upregulated phosphorylated ERK (p-ERK) levels and increased cell proliferation in vitro, but inhibited cell invasion. However, SPRY2 knockdown in SW480 cells (activating KRAS mutant) or Caco-2 cells transfected with KRAS-mutant plasmid did not significantly alter p-ERK levels, cell proliferation, or invasion. The xenografts of SPRY2-knockdown Caco-2 cells were larger with less deep muscle invasion than those of control cells. The clinical cohort study revealed a positive association of SPRY2 protein expression with pT status, lymphovascular invasion, and perineural invasion in KRAS-WT CRCs. However, the associations were not observed in KRAS-mutant CRCs. Interestingly, high SPRY2 expression was related to shorter cancer-specific survival in both KRAS-WT and KRAS-mutant CRC patients. Our study demonstrated the dual role of SPRY2 as an inhibitor of RAS/ERK-driven proliferation and as a promoter of cancer invasion in KRAS-WT CRC. SPRY2 may promote the invasion and progression of KRAS-WT CRC, and might also enhance KRAS-mutant CRC progression through pathways other than invasion.

Evaluation and Application of Drug Resistance by Biomarkers in the Clinical Treatment of Liver Cancer.

Liver cancer is one of the most lethal cancers in the world, mainly owing to the lack of effective means for early monitoring and treatment. Accordingly, there is considerable research interest in various clinically applicable methods for addressing these unmet needs. At present, the most commonly used biomarker for the early diagnosis of liver cancer is alpha-fetoprotein (AFP), but AFP is sensitive to interference from other factors and cannot really be used as the basis for determining liver cancer. Treatment options in addition to liver surgery (resection, transplantation) include radiation therapy, chemotherapy, and targeted therapy. However, even more expensive targeted drug therapies have a limited impact on the clinical outcome of liver cancer. One of the big reasons is the rapid emergence of drug resistance. Therefore, in addition to finding effective biomarkers for early diagnosis, an important focus of current discussions is on how to effectively adjust and select drug strategies and guidelines for the treatment of liver cancer patients. In this review, we bring this thought process to the drug resistance problem faced by different treatment strategies, approaching it from the perspective of gene expression and molecular biology and the possibility of finding effective solutions.

Risk factors combine in a complex manner in assessment for macrosomia.

BACKGROUND: Macrosomia is a serious public health concern. This study aimed to examine the combined effects of various risk factors on macrosomia. METHODS: The China Labor and Delivery Survey was a multicenter cross-sectional study that included 96 hospitals. Logistic regression analysis was performed to examine the combined effects of the risk factors for macrosomia. The population attributable risk percentage (PAR%) was calculated for the risk factors. RESULTS: A total of 64,735 live births, including 3,739 neonates with macrosomia, were used for the analysis. The weighted prevalence of macrosomia was 5.8%. Pre-pregnancy overweight/obesity, diabetes, and gestational hypertension have a synergistic effect on increasing the rate of macrosomia in mothers aged < 36 years. The highest odds ratio (36.15, 95% CI: 34.38-38.02) was observed in female fetuses whose mothers had both gestational hypertension and diabetes. However, in mothers aged ≥ 36 years, the synergistic effect of gestational hypertension and other factors did not exist, and the risk for macrosomia was reduced by 70% in female fetuses of mothers with both gestational hypertension and overweight/obesity. Pre-pregnancy risk factors (pre-pregnancy overweight/obesity and advanced maternal age) contributed the most to macrosomia (23.36% of the PAR%), and the single largest risk factor was pre-pregnancy overweight/obesity (17.43% of the PAR%). CONCLUSION: Macrosomia was related to several common, modifiable risk factors. Some factors have combined effects on macrosomia (e.g., pre-pregnancy overweight/obesity and diabetes), whereas gestational hypertension varies by maternal age. Strategies based on pre-pregnancy risk factors should be given more attention to reduce the burden of macrosomia.

How Children's participation ought to be practiced: A preliminary ethical framework to optimise the participation of children with osteogenesis imperfecta in health care.

BACKGROUND: A three-phase ethnography was conducted at a paediatric orthopaedic hospital exploring the actual and desired participation of children with Osteogenesis Imperfecta in discussions, decisions and actions in the hospital and community. Phase I and Phase II revealed how childhood ethics are understood and practiced in the hospital using artmaking to engage children in discussions about their health-related experiences. Children expressed frustration, anger and disappointment when their desired level of participation in care was not actualized due to forgone opportunities for engagement by clinicians or lack of child-oriented health resources. OBJECTIVES: The objective of this study (Phase III) was to specify how childhood ethics ought to be understood and practiced in the hospital by (1) convening hospital stakeholders in a collaborative setting to disseminate findings, identify ethical concerns and generate action steps; and (2) develop a preliminary ethical framework to optimise the participation of children with OI in health care. DESIGN: Focused ethnography reported using the SRQR checklist. METHODS: A focus group was conducted with 14 interdisciplinary hospital stakeholders. Data were analysed using qualitative, thematic analysis to understand primary ethical concerns and accompanying action steps. The findings were consolidated into a preliminary ethical framework and worksheet for clinicians. RESULTS: Four main factors impeding children's voices and desired participation were identified: legal and contextual factors; variations in clinicians' skills, practice and knowledge; difficulties incorporating alternative engagement methods into practice; and need for interprofessional collaboration. Five action steps were identified: Recognise, Elicit, Interpret, Act and Optimise. RELEVANCE TO CLINICAL PRACTICE: The focus group and creation of clinician resources were important steps towards addressing the ethical concerns of children with OI, such as marginalisation or exclusion in their OI care. This study better prepares us to disseminate our findings on a larger scale and create ethical frameworks and resources to improve how vulnerable children's voices are heard, understood and acted upon in healthcare settings.

Depression in nursing home residents and its correlation with meaning of family involvement and depression of family.

OBJECTIVES: This study aimed to investigate the relationship between depression in older nursing home residents and family caregivers' (FCGs) depressive status and reasons for involvement with residents. DESIGN: This study employed a cross-sectional design. SETTING: Eight nursing homes in northern Taiwan. PARTICIPANTS: A total of 139 older resident-FCG pairs were recruited. MEASUREMENTS: Depression was measured with the Geriatric Depression Scale-Short Form for nursing home residents and the Center for Epidemiologic Studies Depression Scale-Short Form for family members. Depression and demographic data were collected with face-to-face interviews. The meaning ascribed to caregivers' nursing home visits was calibrated using the Family Meaning of Nursing-Home Visits scale. Multiple logistic regression was used to understand the factors related to residents' depressive symptoms. RESULTS: Depressive symptoms were present in 58.3% of the nursing home residents (n = 81). Depressive status of family members (Chi-square = 1.46, p = 0.23) or family's visiting frequency (Chi-square = 1.64, p = 0.44) did not differ between residents with or without depressive symptoms. Factors associated with an increased risk of residents having depressive symptoms were age, self-perceived health status, and having a caregiver motivated to visit to assuage their guilt. CONCLUSIONS: Visiting a family member to assuage their guilt was the only caregiver variable associated with depressive symptoms for nursing home residents. This finding suggests that developing interventions to improve personal relationships between nursing home residents and family members might facilitate the emotional support of caregivers and psychological support for older nursing home residents in Taiwan.

Diagnosis and genetic analysis of a case with glycogen storage disease type V caused by compound heterozygous mutations in the PYGM gene.

Glycogen storage disease type V is an autosomal recessive genetic disorder caused by muscle glycogen phosphorylase (PYGM) deficiency, which is characterized by exercise intolerance, second wind phenomena and high level of serum creatine kinase. In this study, we reported a Chinese young man with glycogen storage disease type V, with lower extremity weakness after exercise, increased creatine kinase, and slight fat infiltration in the posterior group of thigh muscle by magnetic resonance imaging (MRI). The proband had complex heterozygous PYGM disease-causing mutations, including c.308T>C (p.L103P) variant transmitted from the mother and c.260_261delCT (p.S87Ffs*23) from the father, of which the former was a novel PYGM mutation. This study enriched the PYGM pathogenic gene mutation spectrum, contributed to improve clinicians' understanding of glycogen storage disease type V and provided a reference for further genetic study of the disease.

The impact of symptom distress on health-related quality of life in liver cancer patients receiving arterial chemoembolization: the mediating role of hope.

BACKGROUND: Trans-hepatic arterial chemoembolization (TACE) is a treatment option for liver cancer patients. It can prolong patients' survival but can also cause symptom distress. Symptom distress (SDs) can directly impact quality of life (QOL) and may indirectly influence QOL by lessening hope. In this study, we wanted to explore the mediating effect of hope on the relationship between SDs and QOL among patients with liver cancer receiving TACE. METHODS: A cross-sectional study was conducted from December 20, 2017, to August 6, 2018, at a gastroenterology ward of a medical center. The participants were 92 liver cancer patients (69.6% male, mean age 67.8) who were admitted for TACE treatment. Information on SDs, hope, and QOL was collected by questionnaire on discharge day. Hayes' PROCESS model was used to test the mediating effect of hope on the relationship between SDs and QOL. RESULTS: The mean score and standard deviation (SD) of SDs, hope, and QOL were 32.08 (SD = 6.22), 27.09 (SD = 3.51), and 55.16 (SD = 17.33), respectively. SDs negatively impacts quality of life. The total effect of SDs on QOL was - 1.41 (95% confidence interval [CI]: - 1.96, - 0.86). The indirect effect via the mediation of hope was - 0.95 (95% CI: - 1.7, - 0.45). Hope partially mediated the effect of SDs on QOL. CONCLUSION: SDs after TACE is vital; it directly reduces a patient's overall QOL and can indirectly hinder it by reducing the patient's hope. In addition to symptom management, interventions that help patients maintain their hope are key to improving QOL among patients receiving TACE.