Objective:To analyze the efficacy of enzyme replacement therapy and anti-drug antibody production in children with Fabry disease.Methods:The clinical data of 7 children with Fabry disease treated with enzyme replacement therapy for more than 1 year at Children′s Hospital of Zhejiang University School of Medicine from July 2021 to June 2024 were retrospectively analyzed. The basic information and the changes of related clinical indicators before and after treatment were collected. Paired sample t test was used to compare renal function, left heart mass index, pain score and other related indexes before and after treatment. The anti-drug antibodies were detected by enzyme-linked immunosorbent assay. Results:A total of 6 boys and 1 girl were included. The age of diagnosis was (12.2±1.8) years. After 1 year of enzyme replacement therapy, the abnormal substrate globotriaosylsphingosine and brief pain inventory scores of all children were significantly lower than those before treatment ((16±11) vs. (63±42) μg/L, 22±19 vs. 45±29, t=3.88, 3.43, both P<0.05). There were no significant differences in glomerular filtration rate, urinary microalbumin to creatinine and left heart mass index before and after treatment ((124±35) vs. (136±26) ml/(min·1.73 m 2), (9.3±8.3) vs. (3.8±2.5) mg/g, (38±9) vs. (33±6) g/m 2.7, t=1.33, 1.74, 1.19, all P>0.05). Patients 4, 5 and 6 developed anti-drug antibodies at 1 month, 4 months and 1 month after medication, respectively. Patient 4 had persistently high anti-drug antibody titers (absorbance 3.65-3.73) accompanied by urticaria, elevated globotriaosylsphingosine and worsening clinical symptoms. Conclusions:The enzyme replacement therapy can effectively improve the clinical symptoms and reduce the level of globotriaosylsphingosine in children with Fabry disease. The anti-drug antibody is common in patients after long-term enzyme replacement therapy and may diminish the efficacy, which needs dynamic monitoring.

ObjectiveTo investigate the effect and underlying mechanism of the Wulao Qisun prescription on pathological new bone formation in ankylosing spondylitis （AS）. MethodsSynovial fibroblasts were isolated from the hip joints of AS patients and observed under a microscope to assess cell morphology. The cells were identified using immunofluorescence staining. The isolated AS fibroblasts were divided into blank group， low drug-containing serum group， medium drug-containing serum group， high drug-containing serum group， and positive drug group. After drug intervention， cell proliferation was measured using the cell counting kit-8 （CCK-8） assay to observe fibroblast growth and determine the optimal intervention time. Alkaline phosphatase （ALP） activity was measured using the alkaline phosphatase assay. Protein expression of osteocalcin （OCN）， osteopontin （OPN）， and runt-related transcription factor 2 （Runx2） was detected by Western blot. The mRNA expression levels of Wnt5a， β-catenin， and Dickkopf-1 （DKK-1） were measured by real-time quantitative polymerase chain reaction （Real-time PCR）. ResultsCompared with the blank group， each drug-containing serum group of Wulao Qisun prescription and the positive drug group inhibited the proliferation of AS fibroblasts and reduced ALP expression （P<0.01）. Compared with the blank group， the low drug-containing serum group of Wulao Qisun prescription downregulated β-catenin mRNA expression （P<0.05）. The medium and high drug-containing serum groups and the positive drug group significantly downregulated Wnt5a and β-catenin mRNA expression （P<0.05， P<0.01）， with the positive drug group showing the most pronounced effect （P<0.01）. The high drug-containing serum group and the positive drug group significantly upregulated DKK-1 mRNA expression （P<0.01）. Compared with the blank group， the low drug-containing serum group of Wulao Qisun prescription inhibited the expression of OPN and Runx2 proteins （P<0.05， P<0.01）， while the medium and high drug-containing serum groups and the positive drug group inhibited the expression of OCN， OPN， and Runx2 proteins （P<0.05， P<0.01）. ConclusionThe Wulao Qisun prescription can inhibit the proliferation and osteogenic differentiation of AS fibroblasts， thereby delaying the formation of pathological new bone in AS. The possible mechanism involves the regulation of Wnt/β-catenin-related gene expression， further inhibiting the transcription of downstream target genes.

ObjectiveTo improve the quality and service performance of community visual health services in Shanghai, and to establish a set of reasonable and effective evaluation index system for community visual health services. MethodsCentered on the national and Shanghai-based visual health policies and based on the current status and development trends of community visual health service program in Shanghai, the candidate indicators were formed through literature review and expert interviews, firstly. The framework of an evaluation index system was formulated through qualitative research successively, which was further revised and perfected using the Delphi method. Coefficient weights were calculated using the analytic hierarchy process (AHP), culminating in the establishment of the community visual health evaluation index system, lastly. ResultsA total of 22 visual health experts from district-level center for disease control, hospital ophthalmology and leaders in charging of visual health service in community health centers participated in the Delphi questionnaire survey, with a questionnaire recovery rate of 100% and an expert authority coefficient of 0.86, indicating high credibility. After a round of correspondence to experts’ importance ratings and discussions, a comprehensive evaluation index system comprising 3 primary indicators, 12 secondary indicators, and 47 tertiary indicators, along with 5 additional indicators, was finalized. ConclusionAn index system tailored to effective evaluation for community visual health initiatives was drawn up in this study, which can promote the capacity building in community eye health services, facilitating the high-quality development of visual health courses, and enhancing residents’ eye health.

This study explores the diagnosis and treatment of needle knife therapy for ankylosing spondylitis (AS) at middle and advanced stage based on the theory of meridian tendons, from a holistic perspective and syndrome differentiation. The treatment strategy includes "harmonizing yin and yang" to address root causes and "tendons-based release" to harmonize qi and blood, with the "tendons nodule points" as the core acupoint selection criterion. Based on this approach, the study systematically elaborates on two needle knife methods for AS: "governor vessel bone-piercing technique" and "below-the-umbilicus release technique", covering indications, acupoint location, and procedures. Clinical case examples are provided to enrich needle knife therapy guided by the theory of meridian tendons, offering insights for clinical and research work on AS.
Humans ; Acupuncture Points ; Acupuncture Therapy/methods* ; Meridians ; Spondylitis, Ankylosing/physiopathology* ; Tendons/physiopathology*

OBJECTIVE: To assess the impact of albumin (Alb) administration on the prognosis of patients with acute kidney injury (AKI). METHODS: Clinical data of AKI patients in the intensive care unit (ICU) were retrospectively analyzed from the American Medical Information Mart of Intensive Care-IV (MIMIC-IV), including demographic data, acute physiology score (APS), comorbidities, vital signs, laboratory indicators, treatment status, ICU length of stay, and outcome indicators. The main outcome measure is ICU mortality. AKI patients were divided into Alb infusion group and Alb non infusion group based on whether they received Alb treatment. Multiple imputation was used to process missing data and eliminate variables that missing more than 30%. To ensure the stability of the results, propensity score matching (PSM) and inverse probability weighting (IPW) were used to correct the results. Using Kaplan-Meier survival curve and Cox proportional hazards regression model to evaluate the effect of Alb infusion on ICU survival rate in AKI patients. Perform subgroup analysis based on patient age, gender, and comorbidities to evaluate the prognostic effects of Alb on different patient subgroups. RESULTS: A total of 6 390 AKI patients were included, including 1 721 in the Alb infusion group and 4 669 in the Alb non infusion group. After adjusting for key covariates in the Cox regression model, compared with the Alb non infusion group, patients in the Alb infusion group were significantly younger in age, with APS III score, proportion of vasoactive drugs and continuous renal replacement therapy (CRRT) use, sepsis proportion, heart rate, respiratory frequency, aspartate aminotransferase (AST), alanine aminotransferase (ALT), creatinine (Cr), lactic acid (Lac), and arterial partial pressure of carbon dioxide (PaCO₂) levels significantly higher. The proportion of hypertension, myocardial infarction, and congestive heart failure, as well as blood pressure, urine output, platelet count (PLT), and Alb levels were significantly lower. The results of univariate and multivariate Cox regression analysis on the raw data showed that the risk of death in the Alb infusion group was significantly lower than that in the Alb non infusion group [hazard ratio (HR) = 0.69, 95% confidence interval (95%CI) was 0.60-0.80, all P < 0.05]. The results after propensity score matching (PSM) and inverse probability weighting (IPW) processing are consistent with the original data trend (both P < 0.05). The Kaplan-Meier survival curve showed that the cumulative survival rate during ICU stay in the Alb infusion group was significantly higher than that in the Alb non infusion group (24.48% vs. 12.17%, Log-Rank test: χ² = 74.26, P < 0.05). Subgroup analysis shows that Alb infusion has a more significant survival benefit for AKI patients who use vasoactive drugs, have concurrent sepsis, and do not have liver disease. CONCLUSION Albumin infusion can decrease the ICU mortality of AKI patients.
Humans ; Retrospective Studies ; Acute Kidney Injury/mortality* ; Prognosis ; Male ; Female ; Middle Aged ; Aged ; Intensive Care Units ; Albumins/therapeutic use* ; Proportional Hazards Models ; Adult ; Databases, Factual

In traditional clinical teaching of impacted tooth extraction, there are problems including limited observation fields for students, insufficient doctor-patient communication training, inadequate clinical thinking development, and low levels of clinical participation. Based on the core elements of visualizing jaw structure with perspective, formulating clinical strategy, immersing in clinical participation, and cultivating the spirit of caregiving, we developed a virtual reality simulation teaching platform for the clinical decision analysis of impacted tooth extraction. The virtual simulation-based platform can create three different impacted tooth scenarios in 3D, which demonstrates the process of clinical decision analysis of impacted tooth extraction through virtuality-reality interaction, allowing students to immerse in the discovery, analysis, and resolution of the medical and humanistic problems in the process of impacted tooth extraction. The questionnaire survey showed that 81.36% (48/59) of the students believed that the software could improve their clinical thinking ability; 76.27% (45/59) of the students believed that it could help them master the basic process of impacted tooth extraction; and 62.71% (37/59) of the students believed that it could improve their skills of impacted tooth extraction. By providing immersive learning experience, constructive teaching design, and multi-dimensional teaching evaluation, the software achieved the goals of cultivating students' clinical thinking ability and professional literacy in oral and maxillofacial surgery.

Objective In order to clarify the identification path and the followed principles in this kind of pathological-related medical damage identification,explore the prevention and resolution ideas of pathological medical disputes in the medical and health departments,and build a path for the people's court to hear the cases of pathological medical injury liability disputes.Methods Taking the judgement of medical damage liability disputes that took effect in 2019-2023 and published on the judgement document network as a sample,conduct a retrospective analysis in terms of the site of lesion,medical fault,the consequences of the damage,the causal relationship and the degree of cause(degree of participation),the degree of accident classification and responsibility,the selection of appraisal method,and the acceptancde of appraisal opinions.Results From the analysis of the lesion site,there is a high proportion of misdiagnosis in breast,lung,thyroid,skin and uterus;from the analysis of medical fault,58.4%of delayed treatment and 41.6%of overtreatment due to improper selection of treatment plan;from the analysis of the consequences of damage,the consequences of death only account for about 20%.Most of them are in the category of living appraisal;from the analysis of responsibility division,medical institutions bear secondary reasons,accounting for 30.3%;from the analysis of appraisal and acceptance,medical damage identification methods are mostly used,and the court's acceptance of appraisal opinions reaches 92.1%.Conclusion(1)There are more pathological disputes involving women's bodies,and misdiagnosis is the most in the type of fault;(2)Forensic clinical professional appraisers can also participate in the identification of medical damage involving pathological diagnosis;(3)For cases of medical damage liability disputes involving pathological diagnosis and the consequences of death,the cause of death should be identified;(4)Medical damage identification should be in accordance with the principle of peer review.

Objective:To further study the causes of acute occupational poisoning accidents, and to provide scientific basis and decision support for the prevention of accidents in advance.Methods:From September 2022 to May 2023, the literature was searched and 232 cases of acute occupational poisoning cases occurred from 2013 to 2022 were collected. The causal nodes of the accident were determined according to the expert score, and the interpretative structural model (ISM) was used to construct the correlation model between the causal nodes to obtain the hierarchical relationship between the factors. The influence of each causal node on the occurrence of acute occupational poisoning accidents was studied by using Bayesian network (BN), and the relationship and influence among the causal nodes were analyzed by Netica 5.18 software to establish the pre-prevention model of acute occupational poisoning accidents and identify the key causal factors.Results:A total of 23, 203, and 6 cases of significant, large, and medium acute occupational poisoning accidents were included, of which 179, 29, and 24 cases were asphyxiating gas, irritating gas, and mixed gas, respectively. ISM of acute occupational poisoning accidents divided the causal factors into a 7-layer and 3-level hierarchical structure model. Among them, operation conditions, protective measures, ventilation equipment, hidden trouble investigation, emergency management, illegal operation, equipment and facilities, and blind rescue were the direct causes of the occurrence and expansion of accidents. Warning devices, inspection situation, safety education situation, safety operation procedures, and technology in the production process were indirect influences that lead to the occurrence and expansion of accidents. Safety production responsibility system, enterprise supervision and management and government supervision were the deep-rooted influences. BN reasoning showed that the maximum probability causal chain of acute occupational poisoning accidents was as follows: safety production responsibility system→enterprise supervision and management→safety education and training→protective measures→accident occurrence. The key factors leading to the occurrence of acute occupational poisoning accidents were inadequate protective measures, equipment and facility failures, operational errors, ventilation equipment not being used properly and improper emergency management.Conclusion:In the prevention of acute occupational poisoning accidents, it is necessary to correctly use protective measures, test equipment and facilities before operation, operate according to regulations, ensure the normal use of ventilation equipment, and strengthen emergency management, so as to reduce the incidence of acute occupational poisoning accidents.

Objective:To further study the causes of acute occupational poisoning accidents, and to provide scientific basis and decision support for the prevention of accidents in advance.Methods:From September 2022 to May 2023, the literature was searched and 232 cases of acute occupational poisoning cases occurred from 2013 to 2022 were collected. The causal nodes of the accident were determined according to the expert score, and the interpretative structural model (ISM) was used to construct the correlation model between the causal nodes to obtain the hierarchical relationship between the factors. The influence of each causal node on the occurrence of acute occupational poisoning accidents was studied by using Bayesian network (BN), and the relationship and influence among the causal nodes were analyzed by Netica 5.18 software to establish the pre-prevention model of acute occupational poisoning accidents and identify the key causal factors.Results:A total of 23, 203, and 6 cases of significant, large, and medium acute occupational poisoning accidents were included, of which 179, 29, and 24 cases were asphyxiating gas, irritating gas, and mixed gas, respectively. ISM of acute occupational poisoning accidents divided the causal factors into a 7-layer and 3-level hierarchical structure model. Among them, operation conditions, protective measures, ventilation equipment, hidden trouble investigation, emergency management, illegal operation, equipment and facilities, and blind rescue were the direct causes of the occurrence and expansion of accidents. Warning devices, inspection situation, safety education situation, safety operation procedures, and technology in the production process were indirect influences that lead to the occurrence and expansion of accidents. Safety production responsibility system, enterprise supervision and management and government supervision were the deep-rooted influences. BN reasoning showed that the maximum probability causal chain of acute occupational poisoning accidents was as follows: safety production responsibility system→enterprise supervision and management→safety education and training→protective measures→accident occurrence. The key factors leading to the occurrence of acute occupational poisoning accidents were inadequate protective measures, equipment and facility failures, operational errors, ventilation equipment not being used properly and improper emergency management.Conclusion:In the prevention of acute occupational poisoning accidents, it is necessary to correctly use protective measures, test equipment and facilities before operation, operate according to regulations, ensure the normal use of ventilation equipment, and strengthen emergency management, so as to reduce the incidence of acute occupational poisoning accidents.

Peripheral pulmonary lesions(PPL),including peripheral pulmonary nodules,are common lung problems.As the increase of patients with lung nodules,the demand for tissue sampling also increases.Safe and accurate biopsy techniques are very important for patients to identify benign and malignant lesions.Electronic bronchoscopy is one of the biopsy techniques for the diagnosis of PPL in recent decades.Various guiding techniques,such as radial probe endobronchial ultrasound and virtual navigation bronchoscope,have been proved to improve the performance of conventional bronchoscopy.This paper aims to provide an review of the available data on advanced bronchoscopic techniques and explore their application in diagnosing PPL.