Spinocerebellar ataxia type 6(SCA6)is a group of autosomal dominant hereditary diseases characterized by cerebellar ataxia,with the typical feature of genetic anticipation.This study reports a family case of SCA6 to enhance understanding of the disease and improve clinical diagnosis rate.The patient was a 61-year-old male,presented mainly with slurred speech and unsteady gait,accompanied by dizziness,choking on drinking water,numbness of limbs,and insomnia.Evoked potential,nerve conduction studies,skin sympathetic responses,electroencephalogram,dementia rating scale,and other tests showed no significant abnormalities.Head MRI revealed marked cerebellar atrophy,and genetic testing confirmed SCA6.His father exhibited similar symptoms,while his daughter was a carrier of the abnormal gene.The patient received treatment including nutritional support,improvement of ataxia,and rehabilitation training.After a one-year follow-up,there was no significant progression of the disease.

Objective To develop an evaluation system for quality palliative care and provide an evaluation tool for quality palliative care service.Methods A preliminary evaluation system was drafted by using literature review and group discussion,aligning with the U.S.National Consensus Project for Quality Palliative Care(NCP).The system was revised and refined by two rounds of Delphi consultation with 15 palliative care experts(including specialists in clinical practice,nursing management,nursing research and education)from Tier-IIIA hospitals in Guangdong Province.Indicator weights were determined via consensus.Results Both rounds of expert consultation achieved 100.00%response rates.Expert authority coefficient(Cr)was 0.855.The importance scores of the level-1,level-2 and level-3 indicators of the second round of expert consultation were 4.90-5.00,4.80-5.00 and 4.37-5.00,respectively.Coefficients of variation were 0-0.06,0-0.1 and 0-0.19,respectively.The full score ratio ranged between 0.93 and 1.00,0.8 and 1.00,and 0.67 and 1.00.Kendall's W coefficients were 0.214,0.287 and 0.245,respectively(all P<0.01).The nine level-1 indicators were identified as care structure and process,physiological care,psychological care,social care,mental care,cultural care,end-of-life care,ethical care and quality improvement,with the weight coefficients of 0.123,0.153,0.110,0.106,0.098,0.082,0.119,0.092 and 0.117,respectively.The final evaluation system for quality palliative care included 9 indicators in level-1,22 in level-2 and 69 in level-3.Conclusion The evaluation system for quality palliative care developed on the basis of NCP is scientifically innovative and valid in content.Further studies are required to evaluate its validity..

Objective To develop an evaluation system for quality palliative care and provide an evaluation tool for quality palliative care service.Methods A preliminary evaluation system was drafted by using literature review and group discussion,aligning with the U.S.National Consensus Project for Quality Palliative Care(NCP).The system was revised and refined by two rounds of Delphi consultation with 15 palliative care experts(including specialists in clinical practice,nursing management,nursing research and education)from Tier-IIIA hospitals in Guangdong Province.Indicator weights were determined via consensus.Results Both rounds of expert consultation achieved 100.00%response rates.Expert authority coefficient(Cr)was 0.855.The importance scores of the level-1,level-2 and level-3 indicators of the second round of expert consultation were 4.90-5.00,4.80-5.00 and 4.37-5.00,respectively.Coefficients of variation were 0-0.06,0-0.1 and 0-0.19,respectively.The full score ratio ranged between 0.93 and 1.00,0.8 and 1.00,and 0.67 and 1.00.Kendall's W coefficients were 0.214,0.287 and 0.245,respectively(all P<0.01).The nine level-1 indicators were identified as care structure and process,physiological care,psychological care,social care,mental care,cultural care,end-of-life care,ethical care and quality improvement,with the weight coefficients of 0.123,0.153,0.110,0.106,0.098,0.082,0.119,0.092 and 0.117,respectively.The final evaluation system for quality palliative care included 9 indicators in level-1,22 in level-2 and 69 in level-3.Conclusion The evaluation system for quality palliative care developed on the basis of NCP is scientifically innovative and valid in content.Further studies are required to evaluate its validity..

Cemental tear is a rare and indetectable condition unless obvious clinical signs present with the involvement of surrounding periodontal and periapical tissues. Due to its clinical manifestations similar to common dental issues, such as vertical root fracture, primary endodontic diseases, and periodontal diseases, as well as the low awareness of cemental tear for clinicians, misdiagnosis often occurs. The critical principle for cemental tear treatment is to remove torn fragments, and overlooking fragments leads to futile therapy, which could deteriorate the conditions of the affected teeth. Therefore, accurate diagnosis and subsequent appropriate interventions are vital for managing cemental tear. Novel diagnostic tools, including cone-beam computed tomography (CBCT), microscopes, and enamel matrix derivatives, have improved early detection and management, enhancing tooth retention. The implementation of standardized diagnostic criteria and treatment protocols, combined with improved clinical awareness among dental professionals, serves to mitigate risks of diagnostic errors and suboptimal therapeutic interventions. This expert consensus reviewed the epidemiology, pathogenesis, potential predisposing factors, clinical manifestations, diagnosis, differential diagnosis, treatment, and prognosis of cemental tear, aiming to provide a clinical guideline and facilitate clinicians to have a better understanding of cemental tear.
Humans ; Dental Cementum/injuries* ; Consensus ; Diagnosis, Differential ; Cone-Beam Computed Tomography ; Tooth Fractures/therapy*

Objective:To explore the relationship and underlying mechanisms between servant leadership and satisfaction of medical staff in tertiary hospitals, and to provide references for improving satisfaction of medical personnel.Methods:From January to June 2023, a questionnaire survey was conducted among on-duty medical staff at a tertiary hospital in Guangzhou using a simple random sampling method. Data corresponding to four key variables: servant leadership, hospital management level, affective commitment, and satisfaction of medical staff were collected. SPSS 25.0 software was used to perform independent samples t-tests and one-way analysis of variance (ANOVA) to examine group differences, and Pearson correlation analysis was conducted to explore the relationships among multiple variables. Amos 24.0 software was employed to construct a structural equation model to conduct confirmatory factor analysis of the four key variables, analyze potential mediating effects, and use multi-group analysis to examine differences in path parameters and structure among groups. Results:A total of 632 valid questionnaires were obtained. The satisfaction score of medical staff was (4.50±0.66)(maximum score was 5 points). Age, years of work experience, and job category had statistically significant effects on satisfaction of medical staff ( F = 5.799, 6.483, 7.671; P = 0.001). All four key variables were significantly positively correlated ( P<0.001). Servant leadership, hospital management level, and affective commitment all had direct positive effects on satisfaction of medical staff, with path coefficients of 0.207, 0.386, and 0.345, respectively ( P <0.05, critical ratio>1.96). Hospital management level and affective commitment each had independent partial mediating effects between servant leadership and satisfaction of medical staff (path coefficients of 0.353 and 0.067, respectively; P = 0.007, 0.018). They also jointly exerted a chain mediating effect (path coefficient of 0.243, P = 0.013). Differences in path effects among different job categories (clinical doctors, nurses, and administrative support staff) were statistically significant ( χ2 = 43.344, df = 24, P = 0.009). Conclusions:The servant leadership in tertiary hospitals can directly influence the satisfaction of medical staff, as well as indirectly influence it through emotional commitment and hospital management level. Moreover, the mechanisms of influence vary among medical staff of different professional categories. Tertiary hospitals should introduce and promote servant leadership styles, enhance the servant leadership behaviors of management personnel, and strengthen the synergistic effects of servant leadership, hospital management level, and affective commitment. Differential adjustment mechanisms should be implemented for different job categories.

Hepatic stellate cells (HSCs) are the primary fibrogenic cells in the liver, and their activation plays a crucial role in the development and progression of hepatic fibrosis. Here, we report that retinoid X receptor-alpha (RXRα), a unique member of the nuclear receptor superfamily, is a key modulator of HSC activation and liver fibrosis. RXRα exerts its effects by modulating calcium/calmodulin-dependent protein kinase kinase β (CaMKKβ)-mediated activation of AMP-activated protein kinase-alpha (AMPKα). In addition, we demonstrate that K-80003, which binds RXRα by a unique mechanism, effectively suppresses HSC activation, proliferation, and migration, thereby inhibiting liver fibrosis in the CCl₄ and amylin liver NASH (AMLN) diet animal models. The effect is mediated by AMPKα activation, promoting mitophagy in HSCs. Mechanistically, K-80003 activates AMPKα by inducing RXRα to form condensates with CaMKKβ and AMPKα via a two-phase process. The formation of RXRα condensates is driven by its N-terminal intrinsic disorder region and requires phosphorylation by CaMKKβ. Our results reveal a crucial role of RXRα in liver fibrosis regulation through modulating mitochondrial activities in HSCs. Furthermore, they suggest that K-80003 and related RXRα modulators hold promise as therapeutic agents for fibrosis-related diseases.

Spinocerebellar ataxia type 6(SCA6)is a group of autosomal dominant hereditary diseases characterized by cerebellar ataxia,with the typical feature of genetic anticipation.This study reports a family case of SCA6 to enhance understanding of the disease and improve clinical diagnosis rate.The patient was a 61-year-old male,presented mainly with slurred speech and unsteady gait,accompanied by dizziness,choking on drinking water,numbness of limbs,and insomnia.Evoked potential,nerve conduction studies,skin sympathetic responses,electroencephalogram,dementia rating scale,and other tests showed no significant abnormalities.Head MRI revealed marked cerebellar atrophy,and genetic testing confirmed SCA6.His father exhibited similar symptoms,while his daughter was a carrier of the abnormal gene.The patient received treatment including nutritional support,improvement of ataxia,and rehabilitation training.After a one-year follow-up,there was no significant progression of the disease.

Objective:To explore the relationship and underlying mechanisms between servant leadership and satisfaction of medical staff in tertiary hospitals, and to provide references for improving satisfaction of medical personnel.Methods:From January to June 2023, a questionnaire survey was conducted among on-duty medical staff at a tertiary hospital in Guangzhou using a simple random sampling method. Data corresponding to four key variables: servant leadership, hospital management level, affective commitment, and satisfaction of medical staff were collected. SPSS 25.0 software was used to perform independent samples t-tests and one-way analysis of variance (ANOVA) to examine group differences, and Pearson correlation analysis was conducted to explore the relationships among multiple variables. Amos 24.0 software was employed to construct a structural equation model to conduct confirmatory factor analysis of the four key variables, analyze potential mediating effects, and use multi-group analysis to examine differences in path parameters and structure among groups. Results:A total of 632 valid questionnaires were obtained. The satisfaction score of medical staff was (4.50±0.66)(maximum score was 5 points). Age, years of work experience, and job category had statistically significant effects on satisfaction of medical staff ( F = 5.799, 6.483, 7.671; P = 0.001). All four key variables were significantly positively correlated ( P<0.001). Servant leadership, hospital management level, and affective commitment all had direct positive effects on satisfaction of medical staff, with path coefficients of 0.207, 0.386, and 0.345, respectively ( P <0.05, critical ratio>1.96). Hospital management level and affective commitment each had independent partial mediating effects between servant leadership and satisfaction of medical staff (path coefficients of 0.353 and 0.067, respectively; P = 0.007, 0.018). They also jointly exerted a chain mediating effect (path coefficient of 0.243, P = 0.013). Differences in path effects among different job categories (clinical doctors, nurses, and administrative support staff) were statistically significant ( χ2 = 43.344, df = 24, P = 0.009). Conclusions:The servant leadership in tertiary hospitals can directly influence the satisfaction of medical staff, as well as indirectly influence it through emotional commitment and hospital management level. Moreover, the mechanisms of influence vary among medical staff of different professional categories. Tertiary hospitals should introduce and promote servant leadership styles, enhance the servant leadership behaviors of management personnel, and strengthen the synergistic effects of servant leadership, hospital management level, and affective commitment. Differential adjustment mechanisms should be implemented for different job categories.

Chronic Guillain-Barre syndrome, also known as chronic inflammatory demyelinating polyradiculoneuropathy(CIDP), is an immune-mediated demyelinating peripheral neuropathy. This article analyzes the clinical data of a CIDP patient presenting primarily with limb numbness, pain, and weakness. Along with literature review, this study explores the differential diagnosis between CIDP and diabetic peripheral neuropathy in terms of the pathogenesis, clinical manifestations, laboratory tests, and treatment.

177Lu-prostate specific membrane antigen(PSMA)radio-ligand therapy has been approved abroad for advanced prostate cancer and has been in several clinical trials in China.Based on domestic clinical practice and experimental data and referred to international experience and viewpoints,the expert group forms a consensus on the clinical application of 177Lu-PSMA radio-ligand therapy in prostate cancer to guide clinical practice.