Hepatolenticular degeneration is a rare disease,and the number of cases of primary liver cancer occurring on the basis of liver cirrhosis caused by hepatolenticular degeneration is very small.This paper reports a case of hepatolenticular degeneration with primary liver cancer,and then reviews and summarizes current cases of this disease both domestically and internationally.
Humans ; Hepatolenticular Degeneration/complications* ; Liver Neoplasms/complications*

Cancer is the main cause of death,and drug therapy has greatly improved the effectiveness of anti-tumor treatment.However,there are problems such as high adverse reactions and the risk of developing drug resistance after long-term use.There is an urgent need to seek new drug targets.Human antigen R(HuR),as an RNA binding protein,promotes the whole process of tumor occurrence,development and metastasis through post transcriptional regulation of mRNA stability,and HuR is general-ly highly expressed in tumor tissue,making it a new target for an-ti-tumor therapy and a standard for prognosis evaluation.Tradi-tional Chinese medicine formulas and their various chemical components can inhibit tumor proliferation,induce tumor cell ap-optosis,inhibit angiogenesis,suppress immune escape,and re-verse tumor drug resistance by regulating HuR activity.This re-view summarizes the importance of HuR in regulation of tumor progression,as well as analyzes the mechanisms of the antitumor effects through active ingredients of Chinese medicine with the regulation of HuR.It is expected to provide new ideas for tumor therapy and guidance for the development of HuR-targeted anti-tumor drugs.

BACKGROUND: Based on the China-VHD database, this study sought to develop and validate a Valvular Heart Disease- specific Age-adjusted Comorbidity Index (VHD-ACI) for predicting mortality risk in patients with VHD. METHODS & RESULTS: The China-VHD study was a nationwide, multi-centre multi-centre cohort study enrolling 13,917 patients with moderate or severe VHD across 46 medical centres in China between April-June 2018. After excluding cases with missing key variables, 11,459 patients were retained for final analysis. The primary endpoint was 2-year all-cause mortality, with 941 deaths (10.0%) observed during follow-up. The VHD-ACI was derived after identifying 13 independent mortality predictors: cardiomyopathy, myocardial infarction, chronic obstructive pulmonary disease, pulmonary artery hypertension, low body weight, anaemia, hypoalbuminaemia, renal insufficiency, moderate/severe hepatic dysfunction, heart failure, cancer, NYHA functional class and age. The index exhibited good discrimination (AUC, 0.79) and calibration (Brier score, 0.062) in the total cohort, outperforming both EuroSCORE II and ACCI (P < 0.001 for comparison). Internal validation through 100 bootstrap iterations yielded a C statistic of 0.694 (95% CI: 0.665-0.723) for 2-year mortality prediction. VHD-ACI scores, as a continuous variable (VHD-ACI score: adjusted HR (95% CI): 1.263 (1.245-1.282), P < 0.001) or categorized using thresholds determined by the Yoden index (VHD-ACI ≥ 9 vs. < 9, adjusted HR (95% CI): 6.216 (5.378-7.184), P < 0.001), were independently associated with mortality. The prognostic performance remained consistent across all VHD subtypes (aortic stenosis, aortic regurgitation, mitral stenosis, mitral regurgitation, tricuspid valve disease, mixed aortic/mitral valve disease and multiple VHD), and clinical subgroups stratified by therapeutic strategy, LVEF status (preserved vs. reduced), disease severity and etiology. CONCLUSION The VHD-ACI is a simple 13-comorbidity algorithm for the prediction of mortality in VHD patients and providing a simple and rapid tool for risk stratification.

OBJECTIVE: To explore the relationship between serum chloride levels and prognosis in patients with hepatic coma in the intensive care unit (ICU). METHODS: We analyzed 545 patients with hepatic coma in the ICU from the Medical Information Mart for Intensive Care IV (MIMIC-IV) database. Associations between serum chloride levels and 28-day and 1-year mortality rates were assessed using restricted cubic splines (RCSs), Kaplan-Meier (KM) curves, and Cox regression. Subgroup analyses, external validation, and mechanistic studies were also performed. RESULTS: A total of 545 patients were included in the study. RCS analysis revealed a U-shaped association between serum chloride levels and mortality in patients with hepatic coma. The KM curves indicated lower survival rates among patients with low chloride levels (< 103 mmol/L). Low chloride levels were independently linked to increased 28-day and 1-year all-cause mortality rates. In the multivariate models, the hazard ratio ( HR) for 28-day mortality in the low-chloride group was 1.424 (95% confidence interval [ CI]: 1.041-1.949), while the adjusted hazard ratio for 1-year mortality was 1.313 (95% CI: 1.026-1.679). Subgroup analyses and external validation supported these findings. Cytological experiments suggested that low chloride levels may activate the phosphorylation of the NF-κB signaling pathway, promote the expression of pro-inflammatory cytokines, and reduce neuronal cell viability. CONCLUSION Low serum chloride levels are independently associated with increased mortality in patients with hepatic coma.
Humans ; Male ; Female ; Middle Aged ; Intensive Care Units ; Prognosis ; Chlorides/blood* ; Aged ; Coma/blood* ; Adult

Objectives: This study evaluates the efficacy of a new temporomandibular joint (TMJ) capsule suspension technique for stabilizing the TMJ after free fibular flap reconstruction of the mandibular condyle. Patients and Methods: Patients undergoing the TMJ capsule suspension technique during free fibular flap reconstruction after mandibulectomy with condylectomy (study group; n=9) were compared with a control group (n=9). Mandibular movement trajectory and surface electromyographic signals of bilateral masseters were recorded. The neocondyle–disc relationship was examined with magnetic resonance imaging (MRI) at 6 months after surgery. Results: Maximal mouth opening and bilateral marginal movement distances were comparable between the two groups (P>0.05). The asymmetry index of the condyle path length was significantly higher in controls (P=0.02). Bilateral mouth opening trajectories were symmetric in 7 patients and deviated to the affected side in 2 patients in the study group; they deviated to the affected side in all controls. The mean electromyographic values of the masseter on the affected side in resting, maximum bite, and chewing states were comparable between the two groups (P=0.13, P=0.65, and P=0.82, respectively). On MRI at 6 months, the thicknesses of the anterior, medial, and posterior bands and TMJ disc length were similar on the affected and normal sides in the study group (P=0.57, P=0.13, P=0.48, and P=0.87, respectively). Conclusion The proposed TMJ capsule suspension technique could improve postoperative TMJ structure and function after fibular free flap reconstruction following mandibulectomy with condylectomy.

Objective To develop a novel type of transparent simulation manikin as a surgical training model to meet the surgical treatment demand on the medical train.Methods A transparent manikin was developed with the steps of basic data collection,motherboard design and manufacture and module production and assembly.Firstly,basic data collection was carried out with reference to standardized human anatomy and parameters.Secondly,some software such as UG NX7.5 was used to construct the motherboard of the manikin.Finally,module production and assembly were performed with the materials of acrylic,transparent rubber,silicone and hydrogel and the technology of silicone infusion.Results The transparent manikin developed had its anatomy structure close to that of the real body and high visuality for its internal and external components,which simulated a variety of war wounds and thus could be integrated with the surgical training scenarios on the medical train effectively.Conclusion The transparent manikin developed is characterized by high visuality,modularity and blood flow,and meets the demands for surgical training on the medical train.[Chinese Medical Equipment Journal,2025,46(6):111-115]

Objective:To analyze the predictive role of WT1 expression levels pre-and early post-transplantation on relapse and overall survival(OS)in patients with acute myeloid leukemia(AML)undergoing allogeneic hematopoietic stem cell transplantation(allo-HSCT)during their first complete remission(CR1).Methods:A retrospective analysis was conducted on the clinical data of 107 adult AML patients who underwent allo-HSCT during their CR1 at our center between May 2012 and December 2021.The predictive role of bone marrow WT1 expression levels before transplantation and at 3 and 6 months post-transplantation on relapse and OS was explored in combination with relevant clinical factors.Results:The median follow-up time for the 107 patients was 70(range:11-117)months.Among the patients,15 cases died.Kaplan-Meier survial analysis showed that the 3-year overall survival(OS)rate was 85.0％.20 patients experienced relapse,with a median time to relapse of 8(range:0.5-44)months and a l-year cumulative relapse rate of 13.1％.The overall median value of WT1 before transplantation,3 months after transplantation,and 6 months after transplantation was 0.26％(range:0％-23.64％),with an upper quartile value of 0.74％.No statistically significant differences in WT1 expression levels were observed among the pre-transplantation,3-month post-transplantation,and 6-month post-transplantation time points(P=0.227).Univariate analysis showed that patients with WT1 levels＞0.74％at 3 months post-transplantation had a higher 1-year relapse rate(P=0.029)and lower 3-year OS rate(P＜0.001)compared to patients with WT1 levels ≤0.74％.Other significant factors affecting 1-year relapse included stem cell source(P=0.041)and chronic graft-versus-host disease(cGVHD)(P=0.013).For 3-year OS,additional influencing factors were genetic high risk(P=0.048)and stem cell source(P=0.016).Multivariate analysis revealed that WT1 level＞0.74％at 3 months post-transplantation had a trend to affect 1-year relapse rate(HR=3.309,95％CI:0.958-11.431,P=0.058),while the absence of cGVHD was an independent risk factor for 1-year relapse(HR=3.473,95％CI:0.749-16.100,P=0.037).Only WT1 level＞0.74％at 3 months post-transplantation was an independent risk factor for 3-year OS(HR=6.886,95％CI:2.402-19.738,P＜0.001).Conclusion:High WT1 expression level at 3 months post-transplantation in AML patients undergoing allo-HSCT during CR1 affects the 1-year relapse rate and 3-year OS,and is an independent risk factor affecting 3-year OS.These findings suggest that dynamic monitoring of WT1 expression levels has certain value in prognostic assessment of AML patients who received allo-HSCT during CR1.

Radiation-induced injury is a key factor in determining the prognosis of patients undergoing radiotherapy, highlighting the significant clinical importance of developing drugs for radiation prevention and treatment. Especially in oncology, radiation-induced injury remains a pivotal determinant of therapeutic outcomes, because of its direct correlation with normal tissue damage during radiotherapy. Efforts to mitigate or treat such injury are thus paramount in enhancing the overall safety and efficacy of cancer treatment. Novel nanomedicines with prolonged systemic circulation, versatile drug-loading capacities, enhanced tissue retention, and stimuli responsiveness exhibit unique advantages in the treatment and prevention of radiation-induced diseases, as they can be designed based on the specific microenvironment of radiation-damaged tissues, which offers innovative solutions to address the limitations of conventional radioprotectors such as short half-life, poor tissue targeting, and systemic side effects. This review thus aims to provide an overview of recent advance in the design and application of nanomaterials for radiation prevention and treatment. Generally, ionizing radiation damages cells either by inducing DNA double-strand breaks or through the generation of reactive oxygen species (ROS). The resulting oxidative stress would disrupt the structural integrity of cell membranes, proteins, and nucleic acids, leading to apoptosis, chronic inflammation, and systemic effects across multiple systems, including hematopoietic system, gastrointestinal tract, skin, lungs, brain, and heart. Radiation protection strategies focus on scavenging ROS, stimulating cellular repair and regeneration, inducing tissue hypoxia, and inhibiting apoptotic pathways. Recent advances in nanomedicine have introduced novel approaches for targeted and efficient radiation protection and treatment. For radiation-induced hematopoietic injury, nanoparticles can been designed to promote red and white blood cell regeneration while reducing oxidative stress. To address radiation-induced gastrointestinal injuries, nanomaterials enable localized antioxidant delivery and extended intestinal retention, effectively relieving radiation enteritis by scavenging ROS and modulating gut microbiota. For radiation-induced skin injuries, self-assembling peptide hydrogels that mimic the extracellular matrix can serve as effective scaffolds for wound healing. These hydrogels exhibit excellent antioxidant properties, stimulating angiogenesis, and accelerating the recovery of radiation dermatitis. In cases of radiation-induced brain damage, nanoparticles were designed to cross the blood-brain barrier to rescue neuronal damage and protect cognitive function. This review provides an in-depth insight into the mechanisms underlying radiation-induced injuries and highlights how nanomaterial were construtced according to the specific injury. Therefore, nanotechnology endowers durgs with transformative potential for preventing and treating radiation-induced injuries. Despite significant progress in nanomedicine, there are still challenges in long-term biocompatibility, precise targeting of damaged tissues, and scalable manufacturing. In addition, an in-depth understanding of the interactions between nanomaterials and biological systems remains to be covered. Future efforts should focus on optimizing design strategies, enhancing clinical translatability, and ensuring long-term safety, ultimately improving patient outcomes. Besides, expanding research into other radiation-induced diseases, such as radiation-induced ophthalmic disorders and hepatic injuries, may diversify therapeutic options.

Cancer is the main cause of death,and drug therapy has greatly improved the effectiveness of anti-tumor treatment.However,there are problems such as high adverse reactions and the risk of developing drug resistance after long-term use.There is an urgent need to seek new drug targets.Human antigen R(HuR),as an RNA binding protein,promotes the whole process of tumor occurrence,development and metastasis through post transcriptional regulation of mRNA stability,and HuR is general-ly highly expressed in tumor tissue,making it a new target for an-ti-tumor therapy and a standard for prognosis evaluation.Tradi-tional Chinese medicine formulas and their various chemical components can inhibit tumor proliferation,induce tumor cell ap-optosis,inhibit angiogenesis,suppress immune escape,and re-verse tumor drug resistance by regulating HuR activity.This re-view summarizes the importance of HuR in regulation of tumor progression,as well as analyzes the mechanisms of the antitumor effects through active ingredients of Chinese medicine with the regulation of HuR.It is expected to provide new ideas for tumor therapy and guidance for the development of HuR-targeted anti-tumor drugs.

Objective To explore the changes in symptom clusters and core symptoms at different stages in col-orectal cancer patients undergoing postoperative chemotherapy,and to provide a reference for developing precise symptom management strategies in clinical practice.Methods 300 colorectal cancer patients who underwent post-operative chemotherapy at 2 tertiary hospitals in Tianjin from August 2023 to May 2024 were conveniently selected as the study subjects.General information questionnaires and the Chinese version of the MD Anderson Symptom In-ventory for Colorectal Cancer were used to survey patients during the first chemotherapy cycle(T1)and the third chemotherapy cycle(T2).Exploratory factor analysis was used to extract symptom clusters;R language was used to construct symptom networks.Results 300 questionnaires were distributed and collected at T1,and 264 question-naires were distributed and collected at T2,with a recovery rate of 88.00％.Colorectal cancer patients undergoing postoperative chemotherapy had 5 symptom clusters at T1,and 4 symptom clusters at T2.In the symptom network,distress was the symptom with the highest strength(rs=1.188),closeness centrality(rc=0.004),and betweenness centrality(rb=36)at T1.Distress was the symptom with the highest strength(rs=1.397),while decreased appetite had the highest closeness centrality(rc=0.004)and betweenness centrality(rb=39)at T2.Conclusion The symptom clusters in colorectal cancer patients undergoing postoperative chemotherapy remain stable over time.Distress is a stable core symptom during chemotherapy,while decreased appetite is a bridging symptom in the mid-chemotherapy phase.Healthcare providers should prioritize interventions for decreased appetite while addressing emotional symptoms to improve the efficiency of symptom management.