Autosomal dominant polycystic kidney disease （ADPKD） is a systemic hereditary renal disorder and can affect multiple organs， and congenital hepatic fibrosis is one of the manifestations of liver involvement and is an important complication of ADPKD. Symptomatic management is currently the main treatment method for this disease， and disease-specific drugs such as tolvaptan have limited indications and cannot correct the underlying genetic defect. This article reports a case of ADPKD with congenital hepatic fibrosis， and sirolimus was identified as the individualized treatment regimen based on peripheral blood functional genomic analysis and drug sensitivity prediction platform. The patient achieved significant improvements in symptoms and quality of life after treatment， with a stable kidney volume. This case shows that functional genomics has a potential value in guiding individualized treatment of rare genetic disorders， which provides new treatment ideas and practice paths for similar patients.

Pharmacovigilance runs through the whole life cycle of drugs. This paper systematically sorts out the development course of pharmacovigilance in China， from the initial establishment of the adverse drug reaction reporting system to the gradual construction of a complete pharmacovigilance system aligned with international standards. It focuses on analyzing the evolutionary features of the organizational system， legal system and monitoring system during this process. On this basis， this paper further discusses the future development trends of pharmacovigilance in China. In general， remarkable progress has been achieved in both regulatory construction and practical implementation of China’s pharmacovigilance system. In the future， efforts should be continuously made to improve the pharmacovigilance system， such as accelerating the implementation of the “one-body with two-wings” working framework and strengthening the primary responsibility of marketing authorization holders. It is also necessary to cultivate and reserve professional talents and promote information-based construction， including optimizing the spontaneous reporting system and establishing active monitoring systems. These measures can provide more solid support for ensuring public drug safety and facilitating high-quality development of the pharmaceutical industry.

ObjectiveTo explore the mechanism by which the ethanol extract of Epimedium brevicornu （EEBM） intervenes in mesenchymal adipose-derived stem cells （ADSCs） to delay aging in castrated rats. MethodsForty-five 3-month-old SPF female SD rats were ovariectomized and randomly divided into model group， ADSCs treatment group， and ADSCs groups treated with low， medium， and high concentrations of EEBM （1， 50， 100 μg·L^-1）， referred to as the AE low， medium， and high concentration groups， with 9 rats in each group. After tail vein injection of 200 μL of the corresponding stem cell suspension， aging-related indicators including cyclin-dependent kinase inhibitor （p21）， tumor suppressor gene （p53）， interleukin-6 （IL-6）， interleukin-8 （IL-8）， superoxide dismutase （SOD）， malondialdehyde （MDA）， B-cell lymphoma-2 （Bcl-2）， Bcl-2-associated X protein （Bax）， cysteine-aspartic acid protease-3 （Caspase-3）， and lipofuscin were measured using enzyme-linked immunosorbent assay （ELISA） and Western blot. ResultsCompared with the model group， the IL-6 content in the AE low， medium， and high concentration groups was significantly decreased （P<0.05）. Lipofuscin， MDA， and IL-8 levels in the ADSCs treatment group and AE low， medium， and high concentration groups were significantly reduced （P<0.01）， while SOD content was significantly increased （P<0.05， P<0.01）. Compared with the ADSCs treatment group， lipofuscin and IL-8 levels in the AE low， medium， and high concentration groups were significantly reduced （P<0.05， P<0.01）. The MDA content was significantly decreased in the AE medium concentration group （P<0.01）. Compared with the model group， protein levels of p21， p53， Bax， and Caspase-3 in the ADSCs treatment group and AE low， medium， and high concentration groups were significantly reduced （P<0.05， P<0.01）， while the Bcl-2 protein level was significantly increased （P<0.01）. Compared with the ADSCs treatment group， protein levels of p21， p53， Bax， and Caspase-3 in the AE low， medium， and high concentration groups were significantly reduced （P<0.05， P<0.01）， and the Bcl-2 protein level in the AE low concentration group was significantly increased （P<0.01）. ConclusionThe results of this experiment show that EEBM-treated ADSCs or ADSCs may delay aging in castrated rats by inhibiting cell apoptosis， reducing cell cycle inhibitors and pro-inflammatory factors， enhancing antioxidant capacity， and reducing oxidative reactions. Moreover， EEBM-treated ADSCs demonstrate stronger anti-aging effects than ADSCs alone. This study provides experimental evidence supporting the clinical use of EEBM to intervene in ADSCs and delay aging.

Objective To observe the value of the integrated model of MR high-resolution vascular wall imaging(HR-VWI)and attention mechanism for predicting stroke recurrence in symptomatic intracranial atherosclerotic stenosis(sICAS)patients.Methods A total of 363 patients with sICAS who underwent HR-VWI were enrolled and stratified into training set(n=254)and validation set(n=109)according to their origins.Employing a radiomics model that utilized HR-VWI T1 and contrast-enhanced sequences for feature extraction,image data were captured from relevant plaques.Subsequently,a Trans model was developed by integrating the Transformer attention mechanism.The predictive performance and clinical utility of conventional radiomics models and Trans models for forecasting stroke recurrence among patients with sICAS were evaluated.Results In training set and validation set,the area under the curve of Trans model for predicting stroke recurrence in sICAS patients was 0.992 and 0.988,respectively,both superior to that of T1 model,T1 enhanced model and dual sequence model(all P＜0.05).The calibration curve and decision curve analysis showed that Trans model had good predictive probability and clinical practicality.Conclusion The obtained integrated model of HR-VWI radiomics combined with attention mechanism had certain value for predicting stroke recurrence in patients with sICAS.

Objective To observe the value of the integrated model of MR high-resolution vascular wall imaging(HR-VWI)and attention mechanism for predicting stroke recurrence in symptomatic intracranial atherosclerotic stenosis(sICAS)patients.Methods A total of 363 patients with sICAS who underwent HR-VWI were enrolled and stratified into training set(n=254)and validation set(n=109)according to their origins.Employing a radiomics model that utilized HR-VWI T1 and contrast-enhanced sequences for feature extraction,image data were captured from relevant plaques.Subsequently,a Trans model was developed by integrating the Transformer attention mechanism.The predictive performance and clinical utility of conventional radiomics models and Trans models for forecasting stroke recurrence among patients with sICAS were evaluated.Results In training set and validation set,the area under the curve of Trans model for predicting stroke recurrence in sICAS patients was 0.992 and 0.988,respectively,both superior to that of T1 model,T1 enhanced model and dual sequence model(all P＜0.05).The calibration curve and decision curve analysis showed that Trans model had good predictive probability and clinical practicality.Conclusion The obtained integrated model of HR-VWI radiomics combined with attention mechanism had certain value for predicting stroke recurrence in patients with sICAS.

Objective:To investigate the current status of real time application of domestic and imported medical device in the real-world in Liaoning province,and provide data support for the promotion and application of domestic medical device.Methods:Data of the usage of medical device were collected from 77 medical institutions at all levels in 9 urban-rural demonstration regions with representativeness in Liaoning Province from November 2017 to June 2020.Descriptive analysis was used to characterize the distribution of medical device,which data were collected in urban-rural medical institutions at all levels in Liaoning Province.The total amount of receiving test of medical device,and the tested amount of each device in each month in urban-rural medical institutions at all levels were calculated.The median value of monthly average number of persons who received test of various kind of domestic and imported medical device in each month in hospitals with third level and secondary level were also calculated.Results:The total number of device that were collected from 77 medical institutions in 9 demonstration regions in Liaoning province was 325,among of which there were 153 domestic device(accounting for 47.1%)and 172 imported devices(accounting for 52.9%).A total of 6,261,000 person-time valid records were collected from 77 medical institutions,which average test number was 5,562 person-time in each device and each month.The total number of person-times of domestic device in diagnosis and treatment was 1,380,000 person-times(accounting for 22.0%),and that of imported devices was 4,881,000 person-times(accounting for 78.0%).The average test number of each device of domestic device in each month was 2,334 person-times(accounting for 42.0%),and that of imported device in each month was 3,228 person-times(accounting for 58.0%).In urban regions,imported device were dominant,while domestic medical device were dominant in rural regions in Liaoning province.The overall usage frequency of imported medical device was higher in hospitals with third level or secondary level,while the usage frequency of domestic medical device was higher in grassroots medical institutions.Conclusion:There are differences in the real time usage between domestic and imported medical device among 77 medical institutions at all levels in 9 demonstration regions in Liaoning province.Urban regions mainly tend to use imported device,while rural regions more tent to use domestic device.

Objective:To investigate the current status of clinical leadership among Interventional Operating Room nurses in Shandong Province and to analyze its influencing factors.Methods:In December 2024, 220 Interventional Operating Room nurses from 46 ClassⅢ Grade A general hospitals in Shandong Province were selected for a cross-sectional survey using convenience sampling. General Information Questionnaire, Clinical Leadership Survey, Chinese version of Wong and Law Emotional Intelligence Scale (WLEIS-C) , and Jefferson Scale of Empathy-Health Professionals (JSE-HP) were used as survey instruments. Multiple linear regression was used to analyze the factors influencing clinical leadership among Interventional Operating Room nurses. Pearson correlation analysis was used to explore the relationship between emotional intelligence, empathy, and clinical leadership.Results:A total of 220 questionnaires were distributed and 204 valid questionnaires were recovered, with a valid recovery rate of 92.73% (204/220) . The clinical leadership scores of the 204 Interventional Operating Room nurses were (66.35±8.74) , and the dimensions scored, in descending order, as encouraging the heart, enabling others to act, modeling the way, inspiring a shared vision, and challenging the process. The mean scores of WLEIS-C and JSE-HP items were (5.88±0.90) and (4.61±0.63) , respectively. Multiple linear regression analysis showed that the nature of employment and whether or not they participated in leadership training were influencing factors for clinical leadership among Interventional Operating Room nurses (partial regression coefficients of -2.831 and -2.999, respectively; P<0.05) . Emotional intelligence and empathy ability among Interventional Operating Room nurses were positively correlated with clinical leadership ( P<0.05) . Conclusions:Clinical leadership of nurses in the Interventional Operating Room of ClassⅢ Grade A general hospitals in Shandong Province is at a moderate to high level. Nurses who participate in leadership training, have staffing, have greater empathy, and have higher emotional intelligence have greater clinical leadership. It is recommended that nursing administrators add leadership-related courses to the training of nurses in the Interventional Operating Room, and take steps to improve nurses' emotional intelligence and empathy ability, thereby promoting clinical leadership among Interventional Operating Room nurses.

Objective:To investigate the clinicopathological and genetic features of infantile rhabdomyofibrosarcoma (IRFS) with EGFR kinase domain duplication (EGFR-KDD).Methods:The clinical, morphological and immunohistochemical features of three IRFS with EGFR-KDD diagnosed from January 2022 to January 2024 at Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan, China were retrospectively analyzed using PCR or next generation sequencing technique; and related literature was reviewed.Results:There were 1 male and 2 females, aged at presentation ranging from 1 to 4 years. The tumor occurred in the left thigh, right maxillofacial region, and right popliteal space. The presenting symptom was a painless mass which was accidentally discovered. The maximum diameter of tumors ranged from 3 to 5 cm. Microscopically, the tumors were poorly defined and composed of relatively monomorphic spindle cells, arranged in diffuse, fascicular growth patterns, with moderate pale eosinophilic cytoplasm. Mitoses were abundant. A few round rhabdomyoblastic tumor cells with abundant eosinophilic cytoplasm were found. There was no evidence of hemorrhage or necrosis. The tumor cells expressed vimentin, SMA, MSA, desmin, MyoD1 and myogenin; and the Ki-67 proliferation index was 10%-60%. RT-PCR showed EGFR-KDD in all three cases. Gene fusion was detected in three cases based on next generation sequencing, but only one case had EGFR-KDD. Follow-up data for 12 to 36 months showed two patients died of the disease and one patient was alive without recurrences and metastasis.Conclusions:IRFS is a rare soft tissue tumor that resembles infantile fibrosarcoma but has immunohistochemical evidence of rhabdomyoblastic differentiation. It more commonly occurs in infants and tends to appear in limbs and torso with poor prognosis. Aggressive multimodality treatment is recommended for these patients. EGFR-KDD may be a genetic driver to IRFS. Clinical response to EGFR targeted therapy might be promising in the future.

Objective:To investigate the current status of real time application of domestic and imported medical device in the real-world in Liaoning province,and provide data support for the promotion and application of domestic medical device.Methods:Data of the usage of medical device were collected from 77 medical institutions at all levels in 9 urban-rural demonstration regions with representativeness in Liaoning Province from November 2017 to June 2020.Descriptive analysis was used to characterize the distribution of medical device,which data were collected in urban-rural medical institutions at all levels in Liaoning Province.The total amount of receiving test of medical device,and the tested amount of each device in each month in urban-rural medical institutions at all levels were calculated.The median value of monthly average number of persons who received test of various kind of domestic and imported medical device in each month in hospitals with third level and secondary level were also calculated.Results:The total number of device that were collected from 77 medical institutions in 9 demonstration regions in Liaoning province was 325,among of which there were 153 domestic device(accounting for 47.1%)and 172 imported devices(accounting for 52.9%).A total of 6,261,000 person-time valid records were collected from 77 medical institutions,which average test number was 5,562 person-time in each device and each month.The total number of person-times of domestic device in diagnosis and treatment was 1,380,000 person-times(accounting for 22.0%),and that of imported devices was 4,881,000 person-times(accounting for 78.0%).The average test number of each device of domestic device in each month was 2,334 person-times(accounting for 42.0%),and that of imported device in each month was 3,228 person-times(accounting for 58.0%).In urban regions,imported device were dominant,while domestic medical device were dominant in rural regions in Liaoning province.The overall usage frequency of imported medical device was higher in hospitals with third level or secondary level,while the usage frequency of domestic medical device was higher in grassroots medical institutions.Conclusion:There are differences in the real time usage between domestic and imported medical device among 77 medical institutions at all levels in 9 demonstration regions in Liaoning province.Urban regions mainly tend to use imported device,while rural regions more tent to use domestic device.

Objective:To investigate the clinicopathological and genetic features of infantile rhabdomyofibrosarcoma (IRFS) with EGFR kinase domain duplication (EGFR-KDD).Methods:The clinical, morphological and immunohistochemical features of three IRFS with EGFR-KDD diagnosed from January 2022 to January 2024 at Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan, China were retrospectively analyzed using PCR or next generation sequencing technique; and related literature was reviewed.Results:There were 1 male and 2 females, aged at presentation ranging from 1 to 4 years. The tumor occurred in the left thigh, right maxillofacial region, and right popliteal space. The presenting symptom was a painless mass which was accidentally discovered. The maximum diameter of tumors ranged from 3 to 5 cm. Microscopically, the tumors were poorly defined and composed of relatively monomorphic spindle cells, arranged in diffuse, fascicular growth patterns, with moderate pale eosinophilic cytoplasm. Mitoses were abundant. A few round rhabdomyoblastic tumor cells with abundant eosinophilic cytoplasm were found. There was no evidence of hemorrhage or necrosis. The tumor cells expressed vimentin, SMA, MSA, desmin, MyoD1 and myogenin; and the Ki-67 proliferation index was 10%-60%. RT-PCR showed EGFR-KDD in all three cases. Gene fusion was detected in three cases based on next generation sequencing, but only one case had EGFR-KDD. Follow-up data for 12 to 36 months showed two patients died of the disease and one patient was alive without recurrences and metastasis.Conclusions:IRFS is a rare soft tissue tumor that resembles infantile fibrosarcoma but has immunohistochemical evidence of rhabdomyoblastic differentiation. It more commonly occurs in infants and tends to appear in limbs and torso with poor prognosis. Aggressive multimodality treatment is recommended for these patients. EGFR-KDD may be a genetic driver to IRFS. Clinical response to EGFR targeted therapy might be promising in the future.