This article reports a rare case of autoinflammatory disease presenting initially with skin induration and swelling after trauma as the initial manifestation, followed by progressive limb weakness. The patient was a middle-aged female who developed skin induration and swelling after trauma, which gradually progressed to limb weakness, dysarthria and bilateral facial paralysis, accompanied by livedo reticularis of the lower extremities, diffuse skin induration of the limbs, and beaded subcutaneous nodules in the right upper limb. The patient had a susceptibility to infection since childhood and a history of chronic livedo reticularis. Skin pathological examination revealed panniculitis. A comprehensive etiological screening for special infections and autoimmune diseases was completed with an unremarkable results, and whole-exome sequencing showed no abnormal findings. Following a multidisciplinary discussion combined with RNA sequencing results, the patient was diagnosed with an autoinflammatory disease, with a suspected type Ⅰ interferonopathy. Treatment with tofacitinib resulted in gradual improvement of clinical symptoms. This case highlights the importance of detailed medical history collection, systematic physical examination and multidisciplinary collaborative diagnosis and treatment, and underscores the pivotal role of molecular diagnosis in the confirmation of rare diseases. It can provide a reference for the clinical diagnosis and management of similar rare cases.

Objective To develop a Self-Report Scale of Symptoms for Post Intensive Care Syndrome-Family(PICS-F)and validate its reliability and validity,providing a scientific tool for healthcare professionals to comprehensively assess post intensive care syndrome-family.Methods Based on the symptom experience dimension of Symptom Management Theory,a preliminary version of the scale was developed through literature analysis,semi-structured interviews,expert consultations,and a pilot survey.A convenience sampling method was used to conduct the pre-trial version of the scale questionnaire survey on 442 family members of ICU patients at a tertiary A comprehensive hospital in Wuhan from June to September 2024.The test of the validity and reliability was conducted.Results The developed scale consists of 4 dimensions and 20 items.Exploratory factor analysis identified 4 factors,with a cumulative variance contribution rate of 70.921％.The content validity index at the scale level was 0.941,and the content validity indices for individual items ranged from 0.809 to 1.000.Confirmatory factor analysis indicated that the model fit indices were within acceptable ranges.The overall Cronbach's αcoefficient for the scale was 0.941,and the split-half reliability was 0.832.Conclusion The Self-Report Scale of Symptoms for PICS-F demonstrates good reliability and validity.It can comprehensively reflect the primary symptoms of PICS-F,and can serve as an effective measurement tool for clinicians to assess PICS-F in family members of ICU patients.

Objective:To document the effectiveness of ultrasound-guided genicular nerve block (GNB) in treating knee osteoarthritis (KOA).Methods:A total of 92 KOA patients were randomly divided into an observation group and a control group with 46 in each. Those in both groups were treated conventionally, including with non-steroidal anti-inflammatory drugs, acupuncture, ultrasound, laser irradiation and manipulation therapy. The observation group additionally underwent ultrasound-guided genicular nerve block treatment, once a week for 2 weeks. Pain scoring on a visual analog scale (VAS), the Western Ontario and McMaster University Osteoarthritis Index (WOMAC) and a 6-minute walk test (6MWT) were used to evaluate everyone before and after the treatment, and then 8 weeks later.Results:In the observation group the average VAS rating [(3.54±2.00) at week 2 and (4.13±2.04) at week 8] and the average WOMAC subscale and total scores [(36.91±16.91) at week 8] had improved significantly right after the experiment and 8 weeks later. But in the control group this was true only right after the treatment. The observation group also demonstrated superior improvements in 6MWT distance at week 2 [(434.22±125.19)m] and week 8 [(446.35±126.45)m] compared to both its own baseline and the control group.Conclusions:Ultrasound-guided genicular nerve block is a rapid, precise, effective, and long-lasting intervention for alleviating pain, improving knee function and enhancing walking endurance in KOA patients.

Objective To investigate the mechanism of total ginsenosides(TG)in alleviating myocardial injury in septic rat models by regulating solute carrier family 7 member 11/glutathione per-oxidase 4(SLC7 A1 1/GPX4)-mediated ferroptosis.Methods Ninety SPF-grade male rats were ran-domly divided into five groups:Sham group(sham operation),septic cardiomyopathy(SCM)group,TG40 group(SCM model rats gavaged with 40 mg/kg TG),TG160 group(SCM model rats gavaged with 160 mg/kg TG),and TG160+RSL3 group(SCM model rats gavaged with 160 mg/kg TG and intraperitoneally injected with 10 mg/kg ferroptosis inducer RSL3).The SCM model was established using the cecal ligation and puncture method.Cardiac function was assessed in each group.Hematox-ylin-eosin(HE)staining was performed to observe pathological changes in myocardial tissue.Levels of tumor necrosis factor(TNF)-α,interleukin(IL)-1 β,IL-6,creatine kinase-MB(CK-MB),myo-globin(Mb),and cardiac troponin I(cTnI)were measured by enzyme-linked immunosorbent assay.Protein expression levels of NADPH oxidase(NOX)1,NOX2,NOX4,SLC7A11,and GPX4 were detected by western blotting.Levels of Fe2+and total iron in myocardial tissue were also determined.Results Compared with the Sham group,the SCM group exhibited decreased left ventricular ejec-tion fraction(LVEF),left ventricular fractional shortening(LVFS),and protein expression of SLC7A11 and GPX4,along with increased levels of CK-MB,Mb,cTnI,TNF-α,IL-1β,IL-6,Fe2+,total iron,and protein expression of NOX1,NOX2,and NOX4(P＜0.05).Compared with the SCM group,the TG40 and TG160 groups showed dose-dependent increases in LVEF,LVFS,and SLC7A11/GPX4 expression,as well as reductions in CK-MB,Mb,cTnI,TNF-α,IL-1 β,IL-6,Fe,total iron,and NOX1/NOX2/NOX4 expression(P＜0.05).In contrast,the TG160+RSL3 group displayed lower LVEF,LVFS,and SLC7A11/GPX4 expression,and higher levels of the aforementioned biomarkers compared with the TG160 group(P＜0.05).Conclusion TG sig-nificantly suppresses sepsis-induced myocardial inflammation and oxidative stress,improves cardiac function,and mitigates myocardial injury,likely by inhibiting ferroptosis via activation of the SLC7A11/GPX4 signaling pathway.

ObjectiveTo explore the effect of exercise on anxiety and depression in cancer patients during chemotherapy, as well as the optimal exercise dosage. MethodsA PICO framework was constructed, and randomized controlled trials (RCTs) on the effect of exercise on anxiety and depression in cancer patients during chemotherapy were retrieved from databases of PubMed, Web of Science, Cochrane Library, Embase, Medline, CNKI, VIP and Wanfang data, from the establishment to November, 2023. The quality of the literature was evaluated with Cochrane Risk of Bias Tool and Physiotherapy Evidence Database (PEDro) scale. Data were synthesized and analyzed using RevMan 5.3, and the risk of bias was evaluated using Stata 18.0. ResultsA total of 13 RCTs involving 1 340 subjects were included. The scores of PEDro scale were five to eight. Exercise interventions significantly improved anxiety (SMD = -0.70, 95%CI -1.18 to -0.22, P = 0.004) and depression (SMD = -0.89, 95%CI -1.43 to -0.34, P = 0.002) compared to the control group. Subgroup analyses showed that, the exercise effect on anxiety was less than 45 minutes a time (SMD = -0.26, 95%CI -0.46 to -0.05, P = 0.01), more than three times a week (SMD = -0.26, 95%CI -0.46 to -0.05, P = 0.01), and less than twelve weeks (SMD = -0.21, 95%CI -0.36 to -0.07, P = 0.005). For depression, it was less than 45 minutes a time (SMD = -0.69, 95%CI -1.29 to -0.08, P = 0.03), more than three times a week (SMD = -0.69, 95%CI -1.29 to -0.08, P = 0.03), and less than twelve weeks (SMD = -0.52, 95%CI -0.92 to -0.13, P = 0.01). Moderate to high-intensity exercise interventions significantly outperformed the control group in improving anxiety (SMD = -0.21, 95%CI -0.37 to -0.06, P = 0.007) and depression (SMD = -0.21, 95%CI -0.41 to -0.01, P = 0.04). ConclusionExercise interventions can effectively improve anxiety and depression in cancer patients during chemotherapy, and it suggests for high-intensity exercise, less than 45 minutes a time, more than three times a week, and less than twelve weeks.

Since its establishment in 2016, the National Rare Diseases Registry System of China (NRDRS) has accumulated valuable case data and bio-specimen for basic and clinical research on rare diseases in China. However, the emerging challenges in clinical diagnosis and treatment of rare diseases make it unable for data and resource platform to fully meet the diversified needs. Under this backdrop, we have developed a protocol to optimize and upgrade the system based on the core functions of the NRDRS platform. The goal is to leverage intelligent digital technologies to transform NRDRS into a new platform integrating multimodal data and auxiliary diagnostic and treatment functions. It is specified as the development and construction of "one platform and four intelligent tools." Currently, we have upgraded and developed NRDRS platform, intelligent tool for genotype-phenotype analysis of rare diseases, AI-assisted diagnostic tool for rare diseases, remote multidisciplinary diagnosis and teaching tool for rare diseases, drug screening and validation tool for rare diseases. The next step will focus on the promotion of the application of these tools in clinical settings in order to address the issue of severe imbalance in the allocation of resources for the diagnosis and treatment of rare diseases. This article provides an overview of the digital and intelligent upgrades of the NRDRS, the trials in applications in clinical settings, and direction in the future.

Psoriasis is an incurable chronic inflammatory disease that requires new interventions. Here, we found that fibroblasts exacerbate psoriasis progression by promoting macrophage recruitment via CCL2 secretion by single-cell multi-omics analysis. The natural small molecule celastrol was screened to interfere with the secretion of CCL2 by fibroblasts and improve the psoriasis-like symptoms in both murine and cynomolgus monkey models. Mechanistically, celastrol directly bound to the low-density lipoprotein receptor-related protein 1 (LRP1) β-chain and abolished its binding to the transcription factor c-Jun in the nucleus, which in turn inhibited CCL2 production by skin fibroblasts, blocked fibroblast-macrophage crosstalk, and ameliorated psoriasis progression. Notably, fibroblast-specific LRP1 knockout mice exhibited a significant reduction in psoriasis like inflammation. Taken together, from clinical samples and combined with various mouse models, we revealed the pathogenesis of psoriasis from the perspective of fibroblast-macrophage crosstalk, and provided a foundation for LRP1 as a novel potential target for psoriasis treatment.

Objective To explore the construction of a scientific and reasonable performance appraisal and evaluation system for clinical department heads in public hospitals,so as to provide reference for improving the operational efficiency and medical service quality of hospitals.Methods Preliminary construction of the indicator system through the literature research method,using the Delphi method to conduct two rounds of correspondence with 13 experts in related fields,screening and revising the performance appraisal evaluation indicators,and establishing the final indicator system.Results Formation of a performance appraisal and evaluation system for heads of clinical departments that includes 7 one-vote veto indicators,4 first-level indicators,19 medical and 24 surgical second-level indicators and 3 plus indicators,with the authority coefficients of the two rounds of expert consultation at 0.89 and 0.90,and the harmonization coefficients at 0.504 and 0.630,respectively,and with good results in consistency tests for indicators at all levels.Conclusion The performance appraisal and evaluation system for clinical department chiefs has good science and feasibility.It has played a positive role in promoting the connotative construction of the hospital and improving the management efficiency of the hospital,and can provide a reference for hospital management practice.

Objective:To investigate a novel coating technology for artificial blood vessel pipelines, conducting preliminary tests on the mechanical stability, hemocompatibility, and biocompatibility of the coating.Methods:The polyester braided artificial blood vessels produced by Terumo Corporation were subjected to three different experimental treatments and divided into three groups. Model group: The uncoated artificial blood vessels were thoroughly cleaned and freeze-dried. Experimental group: The artificial blood vessels were first immersed in a dopamine solution to form a polydopamine(PDA) coating on their surface, and then further immersed in an earthworm active protein/gelatin(EWAP/GT) solution to create PDA/GT/EWAP-modified artificial blood vessels. Collagen group: The untreated polyester woven artificial blood vessels served as the control. The study detailed the characterization, porosity, water permeability, degradation rate, blood compatibility, and cytotoxicity of the PDA/GT/EWAP-coated artificial blood vessels. Additionally, a 2-week in vivo study was conducted to evaluate the biocompatibility of the PDA/GT/EWAP-coated artificial blood vessels implanted subcutaneously in SD rats.Results:The PDA/GT/EWAP-coated artificial vascular grafts were successfully fabricated. The artificial blood vessels in the PDA/GT/EWAP group exhibited a porosityof(50.53±1.10)%, with water permeability showing a gradual decreasing trend over time. The overall in vitro degradation rate at 4 weeks was(1.83±0.08)%, and the PDA/GT/EWAP group demonstrated favorable mechanical stability. The activated partial thromboplastin time(APTT) of the PDA/GT/EWAP group was(26.30±0.46)s, the thrombin time(TT) was(18.83±0.49)s, the hemolysis rate was(2.15±0.09)%, and the plasma recalcification time(PRT) was(191.00±10.54)s, indicating excellent blood compatibility and anticoagulant properties. MTT assay evaluation of the PDA/GT/EWAP group revealed no significant cytotoxicity. After subcutaneous implantation of vascular samples in rats for 2 weeks, analysis of blood immune parameters and hematoxylin-eosin(HE) staining of the artificial vascular samples showed that the immune response in the PDA/GT/EWAP group exhibited no significant difference compared to the collagen group and was superior to the model group. Conclusion.Conclusion:The PDA/GT/EWAP composite-coated artificial blood vessel demonstrates excellent mechanical properties, good hemocompatibility, biocompatibility, and low cytotoxicity. It also shows remarkable stability. This research offers a new approach for domestic technological breakthroughs in related fields.

Objective:To investigate the related factors for unexpected positive intraoperative cultures (UPC) in patients undergoing aseptic hip revision and the long-term prosthesis survival rate in such patients.Methods:A retrospective cases series analysis was conducted in 397 patients who underwent aseptic hip revision surgery at the Department of Orthopaedics, First Affiliated Hospital of Xinjiang Medical University from January 2012 to December 2021. There were 225 females and 172 males with an age ( M(IQR)) of 58(23) years (range:21 to 89 years) and a body mass index (BMI) of 25(6) kg/m 2 (range:15 to 39 kg/m 2). Based on the culture results of intraoperative specimens, patients were divided into culture-positive group (32 cases) and culture-negative group (365 cases). The clinical data were analyzed including gender, age, BMI, preoperative C-reactive protein, preoperative erythrocyte sedimentation rate, American Society of Anesthesiologists(ASA) classification, preoperative urinary bacteria, preoperative hypoproteinemia; comorbidities of diabetes mellitus, anemia, chronic obstructive pulmonary disease, cerebral infarction; history of allergy to antibiotics, smoking, drinking, previous prosthetic dislocation, previous surgical intervention; and time from symptom onset to admission, duration of surgery, etc. A univariate analysis was performed by Mann-Whitney U orχ2 test and the independent risk factors were identified by including the independent variables with P<0.20 in the univariate analysis in a logistic regression analysis of dichotomous variables. The study outcomes were defined as :(1) re-surgical treatment for any reason; (2)symptoms of infection such as sinus and incision seepage; (3) persistent excessive hip pain.The Kaplan-Meier survival curve was plotted using the study outcome of any cause as the endpoint event, and the log-rank test was used to compare the 10-year survival rate of the prosthesis in the culture-negative group with that in the culture-positive group. Results:Univariate analysis showed statistically significant differences between the culture-negative and culture-positive groups when comparing gender, BMI, ASA classification, and preoperative urinary bacterial results ( χ2=2.368, P=0.124; χ2=-1.648, P=0.098; χ2=14.128, P=0.003; and χ2=7.384, P=0.007). Logistic regression analysis showed that male, ASA classification Ⅳ, and positive preoperative urinary bacteria were independent risk factors for the development of UPC during aseptic hip revision( OR=2.35,95% CI:1.08 to 5.36, P=0.040; OR=37.32,95% CI:1.80 to 1 810.63, P=0.030; OR=4.11,95% CI:1.40 to 11.12, P=0.012). The follow-up time of the 397 patients included in this study was 70 (134) months (range:12 to 146 months). The Kaplan-Meier survival curves showed that the 10-year survival rates of the prostheses in the culture-negative and culture-positive groups were 95.7% and 75.0%, respectively. There was no statistically significant difference in the 10-year survival rate comparing the two groups ( P=0.661). Conclusions:UPC is more likely to occur in patients undergoing hip revision due to noninfectious factors in those who are male, have positive preoperative urinary bacteria, and have an ASA classification of Ⅳ. Intraoperative specimen culture results (negative or positive) do not affect 10-year prosthesis survival rate after hip revision.