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 compare the clinical efficacy of digital 3D technology assisted and traditional one-hole split endoscopic surgery for lumbar spinal stenosis with degenerative scoliosis(LSS-DS).Methods Clinical data of 42 patients with LSS-DS who underwent one-hole split endoscopic surgery from January 2022 to December 2023 were retrospectively analyzed.Among them,20 cases were treated with digital 3D technology assisted one-hole split endoscopic surgery(3D group),and 22 cases were treated with traditional one-hole split endoscopic surgery(traditional group).The operation time,intraoperative blood loss,lateral articular surface preservation rate,Visual Analogue Scale(VAS)of lower extremity pain,Oswestry Disability Index(ODI)and modified MacNab criteria at the last follow-up were compared between the two groups.Results Both groups of patients were operated successfully.The operation time of the 3D group and the traditional group was(73.3±24.0)min and(65.7±23.8)min,respectively,with no significant difference(t=1.020,P=0.314).The intraoperative blood loss was(65.3±14.7)ml and(66.7±17.1)ml,respectively,with no significant difference(t=-0.298,P=0.767).The retention rate of lateral articular surface in the 3D group[(88.2±6.3)%]was significantly higher than that in the traditional group[(82.7±4.6)%,t=3.201,P=0.003].One patient in the traditional group had dural tear and was cured after conservative treatment.There were 3 cases of calf muscle venous thrombosis in the 3D group and 4 cases in the traditional group.All the 7 patients were given anticoagulant therapy and appropriately delayed time of ambulation.In the3D group,20 cases were followed up for6-13 months,with a mean of(8.2±2.0)months;in the traditional group,22 cases were followed up for 6-12 months,with a mean of(7.7±1.7)months.According to the modified MacNab criteria,the excellent and good rate was 95.0%(19/20)in the 3D group and 90.1%(20/22)in the traditional group at the last follow-up,and there was no significant difference between the two groups(Z=-0.311,P=0.756).The VAS score and ODI of the two groups were significantly improved at 3 months and 6 months after operation(all P=0.000),but there was no significant difference between the two groups at each time point(P>0.05).Conclusion The short-term efficacy of digital 3D technology assisted one-hole split endoscopic surgery in the treatment of LSS-DS is definite,and the symptoms of patients are significantly improved.

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 compare the clinical efficacy of digital 3D technology assisted and traditional one-hole split endoscopic surgery for lumbar spinal stenosis with degenerative scoliosis(LSS-DS).Methods Clinical data of 42 patients with LSS-DS who underwent one-hole split endoscopic surgery from January 2022 to December 2023 were retrospectively analyzed.Among them,20 cases were treated with digital 3D technology assisted one-hole split endoscopic surgery(3D group),and 22 cases were treated with traditional one-hole split endoscopic surgery(traditional group).The operation time,intraoperative blood loss,lateral articular surface preservation rate,Visual Analogue Scale(VAS)of lower extremity pain,Oswestry Disability Index(ODI)and modified MacNab criteria at the last follow-up were compared between the two groups.Results Both groups of patients were operated successfully.The operation time of the 3D group and the traditional group was(73.3±24.0)min and(65.7±23.8)min,respectively,with no significant difference(t=1.020,P=0.314).The intraoperative blood loss was(65.3±14.7)ml and(66.7±17.1)ml,respectively,with no significant difference(t=-0.298,P=0.767).The retention rate of lateral articular surface in the 3D group[(88.2±6.3)%]was significantly higher than that in the traditional group[(82.7±4.6)%,t=3.201,P=0.003].One patient in the traditional group had dural tear and was cured after conservative treatment.There were 3 cases of calf muscle venous thrombosis in the 3D group and 4 cases in the traditional group.All the 7 patients were given anticoagulant therapy and appropriately delayed time of ambulation.In the3D group,20 cases were followed up for6-13 months,with a mean of(8.2±2.0)months;in the traditional group,22 cases were followed up for 6-12 months,with a mean of(7.7±1.7)months.According to the modified MacNab criteria,the excellent and good rate was 95.0%(19/20)in the 3D group and 90.1%(20/22)in the traditional group at the last follow-up,and there was no significant difference between the two groups(Z=-0.311,P=0.756).The VAS score and ODI of the two groups were significantly improved at 3 months and 6 months after operation(all P=0.000),but there was no significant difference between the two groups at each time point(P>0.05).Conclusion The short-term efficacy of digital 3D technology assisted one-hole split endoscopic surgery in the treatment of LSS-DS is definite,and the symptoms of patients are significantly improved.

Extensive penile ulcer secondary to granulomatosis with polyangiitis（GPA）is clinically rare. This study reports a 20-year-old male patient with pathologically confirmed GPA-induced penile ulcers. Following control of GPA progression and wound infection management，reconstruction using a scrotal pedicled flap was performed to address the penile skin defect. The follow-up of 1 year results demonstrated favorable flap survival and significant improvement in penile appearance.

Body weight abnormalities, including overweight, obesity, and underweight, have become a dual public health challenge in Chinese adults: overweight and obesity lead to a variety of chronic complications, while underweight increases the risks of malnutrition, sarcopenia, and organ dysfunction. To systematically address these issues, multidisciplinary experts in endocrinology, sports science, nutrition, and psychiatry from various regions have held multiple weight management seminars. Based on the latest epidemiological data and clinical evidence, they expanded the guideline to include assessment and intervention strategies for underweight, in addition to the core content of obesity management. This guideline outlines the etiological mechanisms, evaluation methods, and multidimensional management strategies for overweight and obesity, covering key areas such as diagnosis and assessment, medical nutrition therapy, exercise prescription, pharmacological intervention, and psychological support. It is intended to provide a scientific and standardized approach to weight management across the adult population, aiming to curb the rising prevalence of obesity, mitigate complications associated with abnormal body weight, and improve nutritional status and overall quality of life.

This experiment was conducted to study the effects of replacing feed protein by Herme-tia illucens larvae meal on immune function,intestinal morphology and microflora of Sichuan white geese.A total of 64 healthy 1-day-old Sichuan white geese were randomly allocated into 4 groups with 4 replicates in each group and 4 geese in each replicate,namely the control group,the 2％HILM,4％HILM and 8％HILM groups fed diets contained 0％,2％,4％and 8％of HILM,re-spectively.The experimental period was 40 days.The results showed that compared with the con-trol group,8％HILM increased the levels of serum IgG1,IgG2a and complement C3,and the difference was statistically significant(P＜0.01).4％HILM significantly increased the expression level of CD4(P＜0.05),and 8％HILM significantly increased the expression level of IL-10(P＜0.05).The ratio of villus length to crypt depth(VH/CD)in the jejunum and ileum in 4％HILM group was significantly increased(P＜0.05).The SIgA level of jejunum was significantly increased in all HILM replacement groups(P＞0.05).The abundance of Bacteroides in 4％HILM group were extremely significantly increased(P＜0.01),and the abundance of Bilophila and Bilophila wadsworthia were significantly decreased in all HILM replacement groups(P＜0.05).In conclu-sion,HILM can enhance the immune function of Sichuan white geese,improve the intestinal mor-phology of jejunum and ileum,enhance the local mucosal immunity of jejunum,increase the abun-dance of beneficial bacteria in cecum and decrease the abundance of harmful bacteria in cecum,and protect intestinal health.

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.