Wilson disease （WD） is characterized by marked heterogeneity in clinical phenotype， and it often overlaps with liver diseases （such as cholestatic liver diseases and active hepatitis） and neuropsychiatric diseases， which may easily lead to misdiagnosis or missed diagnosis. This article focuses on the confusing scenarios in clinical practice， reviews the pathophysiological basis of ATPase copper transporting beta （ATP7B） gene dysfunction， and systematically elaborates on the key interpretation points and limitations of ceruloplasmin， total serum copper/non-ceruloplasmin-bound copper， 24-hour urinary copper excretion， D-penicillamine challenge test， hepatic copper quantification， and histopathological assessment across different clinical scenarios. This article also summarizes the potential application of emerging dynamic copper indicators， such as relative exchangeable copper， in diagnosis， family screening， and treatment monitoring. In addition， it discusses the role of ATP7B genetic testing in “gray-zone” cases， difficulties in interpreting variants of uncertain significance， and the features of mutation spectrum in Chinese population， as well as the potential decline in diagnostic performance of the Leipzig scoring system in the context of complex liver diseases. Overall， the diagnosis of WD should not rely on a single indicator， and it is recommended to adopt a multidimensional hierarchical decision-making pathway that integrates phenotype， biochemical tests， dynamic copper indices， tissue/genetic evidence， and scoring systems. Furthermore， key thresholds and workflows should be optimized using real-world data from China， so as to enhance the efficiency of early identification and familial management， thereby improving the long-term prognosis of patients.

ObjectiveTo investigate the prevalence of muscle changes （including sarcopenia and myosteatosis） and related influencing factors in patients with porto-sinusoidal vascular disorder （PSVD）， and to provide a theoretical basis for the early identification， prevention， and intervention of muscle changes in PSVD patients. MethodsA total of 132 PSVD patients who were diagnosed in Nanjing Second Hospital from July 2017 to July 2024 were enrolled as case group， and the hospital staff who underwent physical examination in 2025 were enrolled as healthy control group. Propensity score matching was performed based on age and sex at a ratio of 1∶1. According to muscle status assessed by abdominal CT， the subjects were divided into non-muscle change group， mild muscle change group （myosteatosis alone）， and severe muscle change group （sarcopenia alone or sarcopenia comorbid with myosteatosis）， with the type and severity of muscle change as the exposure factors. General information， laboratory tests， L3-level CT images， and liver biopsy data were collected for the patients in the case group， and general information and CT images were collected for the individuals in the healthy control group. Sarcopenia was diagnosed by measuring skeletal muscle index at the L3 level （<44.77 cm²/m² for men and <32.50 cm²/m² for women）， and myosteatosis was defined by mean muscle attenuation combined with BMI （BMI <24.9 kg/m² with attenuation <41 HU or BMI ≥25 kg/m² with attenuation <33 HU）. Demographic， laboratory， and clinical parameters were compared between the case group and the healthy control group. The independent-samples t test was used for comparison of normally distributed continuous data between groups， and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between groups； the chi-square test or the Fisher’s exact test was used for comparison of categorical data between groups. The univariate and multivariate Logistic regression analyses were used to identify the factors associated with sarcopenia in PSVD. ResultsAmong the 132 patients with PSVD， there were 83 patients with portal hypertension （PH） and 49 patients without PH， and there were significant differences between these two groups in age， albumin， albumin/globulin ratio， leukocyte count， neutrophil count， red blood cell count， platelet count， direct bilirubin， indirect bilirubin， hemoglobin， blood calcium， cholinesterase， total bile acid， triglyceride， total cholesterol， prothrombin time， international normalized ratio， activated partial thromboplastin time， decompensation， gastroesophageal or ectopic varices， bleeding and ascites （all P<0.05）. The analyses after matching showed that compared with the healthy control group， the case group had significantly higher prevalence rates of abnormal muscle structure （43.18% vs 18.94%， P<0.001）， mild muscle changes （22.73% vs 7.58%， P<0.001）， and severe muscle changes （20.45% vs 11.36%， P<0.001）. Further comparison showed that there was no significant difference in the proportion of patients with muscle changes between the PSVD patients with PH and those without PH （42.17% vs 44.90%， P=0.760）. The binary Logistic regression analysis with the presence or absence of muscle changes as the dependent variable showed that age （odds ratio ［OR］=1.05， 95% confidence interval ［CI］： 1.02 — 1.09， P<0.05）， subcutaneous fat index （OR=1.03， 95%CI： 1.01 — 1.06， P<0.05）， hemoglobin （OR=0.97， 95%CI： 0.95 — 0.99， P<0.05）， and thrombin time （OR=1.26， 95%CI： 1.06 — 1.49， P<0.05） were independent influencing factors for muscle changes in PSVD patients. The multivariate ordinal Logistic regression analysis with the severity of muscle changes as the dependent variable showed that age （OR=1.04， 95%CI： 1.01 — 1.07， P<0.05） and thrombin time （OR=1.17， 95%CI： 1.01 — 1.36， P<0.05） were independent risk factors for the grading of muscle changes. ConclusionMuscle changes are common in PSVD patients， and these changes may be caused by PSVD itself rather than PH. Age， fat distribution， thrombin time， and hemoglobin are important influencing factors for muscle changes.

Objective To investigate the effect of pneumoperitoneum pressure during robotic-assisted kidney transplantation (RAKT) on the function of the transplant kidney. Methods The data of 243 kidney transplant recipients were retrospectively analyzed and divided into open kidney transplantation (OKT) group (n=105) and RAKT group (n=138). The RAKT group was further divided into 13 mmHg group (n=67) and 7 mmHg group (n=71) based on pneumoperitoneum pressure. The donor information, recipient's preoperative general data, intraoperative data, and postoperative recovery of the three groups were compared. In the RAKT group, the renal artery, segmental artery, interlobar artery, and venous flow velocity of the transplant kidney were measured using laparoscopic ultrasound. Results There was a statistically significant difference in donor types among the groups (P<0.05), while other donor information and recipient's preoperative general data showed no statistically significant differences (all P>0.05). There were no statistically significant differences in serum creatinine and complications at 30 days and 1 year postoperatively among the groups (all P>0.05). The OKT group and 7 mmHg group had more intraoperative urine output than the 13 mmHg group. Both RAKT groups had less intraoperative blood loss and shorter hospital stays than the OKT group, and longer operation times than the OKT group (all P<0.05). There were no statistically significant differences in operation time, intraoperative blood loss, and hospital stay between the two RAKT groups (all P>0.05). The vascular flow velocity of the transplant kidney decreased at 13 mmHg compared to 7 mmHg pneumoperitoneum pressure, but the differences were not statistically significant (all P>0.05). Conclusions Controllable pneumoperitoneum pressure has a limited impact on the vascular flow velocity of the transplanted kidney. RAKT is a safe and effective surgical method under appropriate pneumoperitoneum pressure, and choosing a lower pneumoperitoneum pressure is more conducive to the early recovery of renal function postoperatively.

[Objective]To establish a risk recognition model for coronary artery stenosis by using a machine learning method and to identify the key causative factors.[Methods]Patients aged≥18 years,diagnosed with coronary heart disease through coronary angiography from January 2013 to May 2020 in two prominent hospitals in Shanxi Province,were continuously enrolled.Logistic regression,back propagation neural network(BPNN),and random forest(RF)algorithms were used to construct models for detecting the causative factors of coronary artery stenosis.Sensitivity(TPR),specificity(TNR),accuracy(ACC),positive predictive value(PV+),negative predictive value(PV-),area under subject operating characteristic curve(AUC),and calibration curve were used to compare the discrimination and calibration performance of the models.The best model was then employed to predict the main risk variables associated with coronary stenosis.[Results]The RF model exhibited superior comprehensive performance compared to logistic regression and BPNN models.The TPR values for logistic regression,BPNN,and RF models were 75.76%,74.30%,and 93.70%,while ACC values were 74.05%,72.30%,and 79.49%,respectively.The AUC values were:logistic regression 0.739 9;BPNN 0.723 1;RF 0.752 2.Manifestations such as chest pains,abnormal ST segments on ECG,ventricular premature beats with hypertension,atrial fibrillation,regional wall motion abnormalities(RWMA)by color echocardiography,aortic regurgitation(AR),pulmonary insufficiency(PI),family history of cardiovascular diseases,and body mass index(BMI)were identified as top ten important variables affecting coronary stenosis according to the RF model.[Conclusions]Random forest model shows the best comprehensive performance in identification and accurate assessment of coronary artery stenosis.The prediction of risk factors affecting coronary artery stenosis can provide a scientific basis for clinical intervention and help to formulate further diagnosis and treatment strategies so as to delay the disease progression.

Objective To observe the effects of electroacupuncture auricular acupuncture points on depression-like behaviors and ventral dorsal tegmental area(VTA)TH,DRD1 and DRD2 induced in well acute stress disorder(ASD)mice.Methods Experiment 1:18 male C57 mice were divided into 9 control and 9 model mice.Behavioural paradigms such as forced swimming test(FST)and tail suspension test(TST)were used to detect depression-like behaviors in the mice starting on day 5 and day 36;double-labelled staining for immediate early gene protein(C-Fos)and tyrosine hydroxylase(TH)was detected in the VTA of the mice by immunofluorescence.Experiment 2:45 male C57 mice were divided into 15 mice each in the control groups,model groups and electroacupuncture groups.30 days after completion of the electroacupuncture intervention,the mice were tested for depressive behaviors using behavioural paradigms such as FST and TST;dopamine receptor D1(DRD1)and dopamine receptor D2(DRD2)was detected in the VTA of mice in each group using fluorescence quantitative PCR and Western blot techniques;the number of TH neurons in the brain area of the VTA of mice was observed by immunofluorescence.Results Experiment 1:Compared with the control group,the immobile duration of the model group increased in the TST(P<0.001)and FST(P<0.001)on day 5 and day 6,and the immobile duration of the model group increased in the TST(P<0.01)and FST(P<0.01)on day 36 and day 37;compared with the control group,the number of C-Fos and TH fluorescence double-labelled neurons in the model group increased(P<0.05).Experiment 2:At day after completion of the electroacupuncture intervention,the immobile duration of TST(P<0.01)and FST(P<0.01)were decreased in the electroacupuncture group compared with the model group;compared with the control group,DRD1 mRNA(P<0.001)and protein expression(P<0.01)were increased in the VTA of the electroacupuncture group,and DRD2 mRNA(P<0.001)and protein expression(P<0.05)were decreased,TH protein expression(P<0.05)increased,and the number of TH neurons increased(P<0.01).Conclusion Early intervention with auricular electroacupuncture points has an ameliorative effect on depression-like behaviors induced by acute stress disorder in mice,which may be achieved by modulating the activity level of dopaminergic neurons and the expression level of DRD1 and DRD2 in the VTA.

OBJECTIVES: Lymph node metastasis in papillary thyroid cancer (PTC) is closely associated with tumor recurrence and patient survival. However, current technologies have limited sensitivity in detecting occult cervical lymph node metastases. Identifying accurate molecular markers for predicting PTC metastasis holds significant clinical value. This study aims to analyze WNT10A expression in PTC and its clinical significance, and to explore the role of WNT10A gene knockdown in PTC cell proliferation, invasion, and metastasis. METHODS: The expression of WNT10A in thyroid carcinoma was analyzed using the Gene Expression Profiling Interactive Analysis (GEPIA) and University of Alabama at Birminghara Cancer data analysis Portal (UALCAN) databases. Real-time RT-PCR was used to measure WNT10A mRNA levels in tumor and adjacent normal tissues from 32 PTC patients. Immunohistochemistry was conducted on 158 PTC specimens to assess WNT10A protein expression and its correlation with clinicopathological features. In vitro experiments were performed using K1 and TPC-1 cell lines. Cell proliferation was assessed using the Celigo system and methyl thiazolyl tetrazolium (MTT) assays; apoptosis was measured via flow cytometry; invasion and metastasis were evaluated using scratch and Transwell assays. A xenograft model was established in nude mice to observe tumor growth, and tumor weight and volume were compared between cell lines. Differentially expressed genes regulated by WNT10A were identified via mRNA sequencing, followed by Gene Ontology (GO) and ingenuity pathway analysis (IPA). Real-time PCR and Western blotting were used to validate the effects of WNT10A on key downstream mRNA and protein in the Tec kinase signaling pathway. RESULTS: WNT10A mRNA expression was significantly higher in thyroid cancer tissues compared to adjacent normal tissues according to GEPIA and UALCAN (both P<0.01). The real-time RT-PCR result showed that WNT10A mRNA expression in PTC tissues was high than that in adjacent tissues (P<0.01). Immunohistochemistry revealed significantly higher WNT10A protein expression in PTC tissues compared to adjacent tissues (P<0.01), and its expression correlated with multifocality, extrathyroidal invasion, and lymph node metastasis. WNT10A knockdown significantly inhibited proliferation, altered cell cycle distribution, and increased apoptosis in K1 and TPC-1 cells (all P<0.01). WNT10A silencing also reduced migration and invasion abilities in both cell lines. In vivo, WNT10A knockdown in TPC-1 cells suppressed tumor formation in nude mice. GO analysis and IPA suggested that the Tec kinase signaling pathway was a key downstream target of WNT10A. RT-PCR and Western blotting confirmed that WNT10A knockdown downregulated the expression of key genes (STAT3, MAPK8, TNFRSF21, and AKT2) in this pathway. CONCLUSIONS WNT10A is highly expressed in PTC and is associated with tumor proliferation, invasion, and metastasis. Its tumor-promoting effects may be mediated through suppression of the Tec kinase signaling pathway.
Humans ; Cell Proliferation ; Thyroid Cancer, Papillary/pathology* ; Thyroid Neoplasms/metabolism* ; Animals ; Wnt Proteins/metabolism* ; Neoplasm Invasiveness ; Mice ; Cell Line, Tumor ; Female ; Male ; Mice, Nude ; Apoptosis ; Lymphatic Metastasis ; Middle Aged ; Cell Movement ; Adult

Objective To explore the perioperative therapeutic effects and follow-up manage-ment in 16 patients with left ventricular assist device(LVAD)implantation.Methods A retrospec-tive analysis was conducted in data of 16 patients who underwent LVAD implantation in the depart-ment of cardiovascular surgery.Data of 6-minute walk test(6MWT),European Quality of Life-5 Di-mension-5 Levels(EQ-5D-5L),New York Heart Association(NYHA)classification,echocardio-graphy,chest radiography,cardiothoracic ratio,and occurrence of complications(infection,bleed-ing,thrombosis,right heart failure,neurological issues)were collected via the electronic medical re-cord system before surgery and at 30,60,and 90 days postoperatively.Results All patients sur-vived with the pump at 90 days postoperatively.One patient with preoperative renal insufficiency un-derwent dialysis and received a heart transplant after 8 months.One patient developed a sterile granu-loma at the percutaneous lead site on the abdominal wall,which improved after treatment,no complica-tions occurred in other patients.At 90 days postoperatively,there was no statistically significant differ-ence in the right ventricular area change fraction and tricuspid annular plane systolic excursion com-pared with preoperative values(P＞0.05).The left ventricular ejection fraction,left ventricular end-diastolic diameter,and cardiothoracic ratio showed significant improvement compared with preoperative levels(P＜0.05).At 30 days postoperatively,30％of patients recovered to NYHA class Ⅰ and 70％to class Ⅱ;at 60 days,80％of patients to class Ⅰ and 20％to class Ⅱ;at 90 days,90％to classⅠ and 10％to class Ⅱ.The 6MWT and EQ-5D-5L scores of patients significantly increased within 90 days postoperatively(P＜0.01).Conclusion Through rigorous preoperative assessment by a multidisciplinary LVAD team,refinement of surgical techniques,and comprehensive management during hospitalization,self-management before discharge,daily follow-up after discharge,and fol-low-up management upon returning to the hospital for patients with LVAD implantation,the cardiac function and quality of life of patients are significantly improved at 90 days postoperatively.

Objective:To assess the effectiveness of Buzzy? device in alleviating pain and anxiety in children during puncture-related operation,and to provide more comprehensive intervention option for puncture-related management of children.Methods:Randomized controlled trials(RCTs)about the application of Buzzy? device in puncture-related pain and anxiety of children were searched from China National Knowledge Infrastructure(CNKI),Wanfang,VIP,PubMed,Embase and Web of Science(WOS)databases.The retrieval time was from the establishment of the database to July 2023.In the interventions measures,the control groups used different methods that included standard care group,ShotBlocker device group and distraction card group.The obtained 528 relevant literatures were implemented literature selection and data extraction by two independent reviewers according to the established inclusion and exclusion criteria.RevMan5.4 and Stata17.0 software were adopted to conduct Meta-analysis for the pain and anxiety of main outcome indicators,and the success rate of the first puncture,parents'satisfaction and adverse events of secondary outcome indicators in the literatures.Results:A total of 20 eligible studies that could meet the conditions were included,involving 2 876 children,including 1 110 children in the Buzzy? device group and 1 766 children in the control group.The Buzzy? device group was better than the standard care group and the distraction card group in alleviating puncture-related operation pain in children,and the differences of that among them were statistical significance(Z=7.18,2.13,P<0.05),and was better than the standard care group in anxiety,and the difference was statistical significance(Z=5.00,P<0.05).The Buzzy? device group and the distraction card group were better than standard care group and Shotblocker device group in satisfaction,and the difference was significant(x2=63.236,P<0.05).The interventions in the Buzzy? device group and other groups did not have effect on the success rate of the first puncture and incidence of adverse event.Conclusion:The Buzzy? device can alleviate pain and anxiety of children during puncture operation,and improve children's satisfaction for puncture operation.It does not affect the success rate of puncture,and there is not adverse events in its operation.

[Purpose]To analyze the changing trends of the disease burden of liver cancer related to hepatitis B virus(HBV)globally and in China from 1992 to 2021.[Methods]Based on the Global Burden of Disease database in 2021,indicators such as the age-standardized incidence rate and mortality rate of HBV-related liver cancer globally and in China from 1992 to 2021 were collected.The Joinpoint regression model was used to analyze the changing trends of epidemiologi-cal characteristics,and the age-period-cohort model was adopted to analyze the impacts of age,period,and cohort factors on the incidence and mortality risks of HBV-related liver cancer globally and in China.[Results]From 1992 to 2021,the age-standardized incidence rate and age-standardized mortality rate of HBV-related liver cancer globally generally showed a trend of decreasing.The average annual percentage changes were-0.31％and-0.61％,respectively,and all the down-ward trends were statistically significant(both P＜0.05).During the same period,the average annu-al percentage changes of the age-standardized incidence rate and age-standardized mortality rate of HBV-related liver cancer in China were-0.45％and-0.90％,respectively,and all the downward trends were statistically significant(both P＜0.05).The results of the age-period-cohort model anal-ysis showed that from 1992 to 2021,the annual net drift rates of the incidence of HBV-related liver cancer globally and in China were-0.71％(95％CI:-0.84％～-0.57％)and-0.73％(95％CI:-1.01％～-0.44％),respectively.The annual net drift rates of the mortality were-1.15％(95％CI:-1.28％～-1.02％)and-1.42％(95％CI:-1.69％～-1.14％),respectively,all showing an over-all decline.The age effect showed that the risk of HBV-related liver cancer incidence in both the global and Chinese populations began to increase after 30 years of age,peaking in the 70～74 age group,while the risk of mortality surged after 40 years of age and peaked in the population aged 80 and above.The period effect indicated that the incidence and mortality risks were the highest from 1997 to 2001 and the lowest from 2017 to 2021.The cohort effect revealed that the inci-dence and mortality risks gradually decreased in populations born after 1962,with the 2007-2011 birth cohort having the lowest risks.The results of the Wald x2 test showed that there were statistically significant differences in the changing trends of the age,period,and cohort effects on the incidence and mortality of HBV-related liver cancer globally and in China(all P＜0.05).[Con-clusion]From 1992 to 2021,the age-standardized incidence rate and age-standardized mortality rate of HBV-related liver cancer showed a downward trend both globally and in China.The disease burden of HBV-related liver cancer in China was higher than the global level.The age-period-cohort model has revealed the historical changes in the incidence and mortality of HBV-related liver cancer.The current and future situation of the disease burden of HBV-related liver cancer is not optimistic.It is recommended to implement precise stratified interventions for populations of different ages,periods,and birth cohorts,and actively transform the prevention,treatment,and management strategies for HBV-related liver cancer.

Objective:To observe the clinical efficacy of unilateral biportal endoscopy (UBE) assisted bilateral decompression in the treatment of lumbar disc herniation with imaging herniation on one side and clinical symptoms on the contralateral side.Methods:A total of 20 patients with lumbar disc herniation with imaging herniation on one side and clinical symptoms on the contralateral side treated with UBE from January 2022 to January 2024 in the Affiliated Hospital of Binzhou Medical University were retrospectively analyzed. There were 9 males and 11 females, aged 50.4±14.0 years (range, 23-72 years). The intervertebral disc herniation level included L 3-4 in 1 case, L 4-5 in 15 cases, and L 5S 1 in 4 cases. There were 10 cases on the left side and 10 cases on the right side. The duration of symptoms was 24.1±33.7 months (range, 1-120 months). Visual analogue scale (VAS) and Oswestry disability index (ODI) were used to evaluate postoperative pain relief and functional recovery. The clinical efficacy was evaluated by modified MacNab criteria at 6 months after operation. Results:All patients successfully completed the operation. The operation time was 90.3±24.6 min (range, 55-134 mins). The VAS scores of patients at 3 days, 1 month, 3 months and 6 months after operation were 3.6±0.9, 2.2±0.7, 1.2±0.5 and 1.0±0.6, respectively, which were lower than those before operation (6.4±0.8), and the differences were statistically significant ( F=668.728, P<0.001). The ODI at 3 days, 1 month, 3 months and 6 months after operation were 34.2%±4.7%, 28.7%±2.8%, 24.3%±2.1% and 20.5%±2.0%, respectively, which were lower than 69.4%±5.2% before operation, and the differences were statistically significant ( F=515.578, P<0.001). The clinical efficacy was evaluated by modified MacNab criteria at 6 months after operation. Among 20 patients, 18 cases were excellent, 1 case was good, and 1 case was fair. All patients were followed up for 9.1±2.1 months (range, 6-14 months). One patient had a dural tear during the operation, but the range was small and there was no defect, and no further treatment was performed. Numbness of the lower limbs occurred 1 day after operation, and the symptoms disappeared after symptomatic treatment. There was no recurrence of lower limb symptoms, lumbar instability, intervertebral space infection or other complications at the last follow-up. Conclusion:Bilateral decompression with UBE is effective in the treatment of lumbar disc herniation with imaging herniation on one side and clinical symptoms on the contralateral side, which can improve the lumbar pain and function of patients.