Objective:Primary sclerosing cholangitis (PSC) is a rare autoimmune disease. This study aims to describe the baseline characteristics and clinical outcomes of Chinese PSC patients and explore risk factors associated with prognosis, addressing the lack of long-term prognostic analysis in China.Methods:Clinical data of PSC patients were retrospectively collected from May 2009 to June 2023 in Beijing Friendship Hospital Affiliated to Capital Medical University, and patient follow-up was conducted through outpatient visits, telephone calls, and medical record reviews. The Cox proportional hazards model and the Kaplan-Meier method were employed to identify risk factors and estimate transplant-free survival.Results:A total of 65 PSC patients were enrolled, with male patients accounting for 50.8% and an average age of onset of 44 years. The disease types primarily included large duct PSC (57.9%) and whole duct PSC (22.8%). Most patients (78.5%) sought medical attention due to symptoms, with common clinical manifestations including jaundice (32.3%), fatigue (23.1%), abdominal discomfort (21.5%), pruritus (16.9%), and fever (10.8%). A total of 19 patients (29.2%) had concomitant ulcerative colitis. Compared to large duct PSC or whole duct PSC, small duct PSC showed a lower proportion of concomitant ulcerative colitis ( P<0.001) and milder baseline disease severity. After a median follow-up of 29 months (interquartile range: 11,53), 19 patients experienced liver transplantations and/or liver disease-related deaths. The overall 2-year and 5-year transplant-free survival rates for PSC patients were 76.0% and 59.5%, respectively. Elevated bile acid levels were identified as an independent risk factor for poor outcomes in PSC patients. Conclusion:The study population of Chinese PSC patients predominantly consisted of middle-aged males, characterized by a low ratio of asymptomatic cases, a low incidence of associated inflammatory bowel disease, and a low rate of transplant-free survival. Elevated bile acid level was identified as an independent risk factor for poor outcomes in PSC patients.

Objective:To compare the influence of definition methods of different isocenter on postoperative volumetric modulated arc therapy (VMAT) plans for patients with high-grade glioma (HGG),and to explore the feasibility of determining the treatment isocenter at location stage of computed tomography (CT) simulation system (CT-Sim). Methods:A total of 30 HGG patients who received radiotherapy after surgery in Peking University International Hospital from September 1,2021 to December 31,2022 were selected. 4 kinds of methods,which included center of mass (CT-COM) of target region of tumor bed was delineated by CT-Sim location stage,geometric center of target region of treatment plan system (TPS-Geo),whole brain geometric center of treatment plan system (TPS-Head) and center of mass of target region of TPS (TPS-COM),were respectively adopted to definite treatment isocenter for each patient,and they were respectively set as CT-COM group,TPS-Geo group,TPS-Head group and TPS-COM group to design 4 groups of VMAT treatment. The parameters included dose,conformity index (CI),uniformity index (HI) and machine unite (MU) among 4 groups of target region and organ at risk (OAR) were compared. Results:The doses of target region and OAR of four groups were similar,and all of them can meet the requirements of clinical dosimetry and clinical OAR dose limitation,without statistically significant differences (P>0.05). The covered volumes that brain tissues received 10,20,30,40 and 50 Gy doses in CT-COM group,TPS-Geo group and TPS-COM group closed to the maximum dose,the maximum dose of 2％ volume and mean dose (Dmax,D2％,Dmean) of brainstem,bilateral lens,optic chiasm,bilateral optic nerve and pituitary gland. The V10,V20,V30 of brain tissue,and Dmax,D2％,and Dmean of brainstem in TPS-Head group were slightly higher than those in other three groups,and the Dmean of lens,and Dmax,D2％ and Dmean of optic nerve at health side of TPS-Head group were slightly lower than those of other three groups,without statistically significant differences (P>0.05). Conclusion:The four VMAT plans of defining the treatment isocenter can meet the clinical dosimetric requirements,and can determine the isocenter at CT-Sim location stage,which can effectively avoid the occurrence of errors in the reduction of radiotherapy,and increase precision of delivery of radiotherapy dose,and enhance effective utilization rate of equipment.

Objective:To investigate the construction and existing problems of radiotherapy departments in non-public hospitals in Beijing, and to propose improvement suggestions.Methods:An electronic questionnaire survey of 2023 medical education and research situation of the radiotherapy departments of 8 non-public hospitals in Beijing was conducted from March 2024 to June 2024, and on-site quality control supervision and inspection were conducted. The survey covered the equipment allocation, the educational background, professional title, age, working experience of radiotherapy-related personnel, and the operation of the hospital, etc. The questionable questionnaires were reviewed by telephone. The quality control method of entry was double entry and cross-checking. Frequency and composition ratio were used for statistical description. Results:The radiation oncology departments of 8 non-public hospitals in Beijing were mainly located in the main urban areas and suburbs. There were 105 radiotherapy practitioners, including 37 radiation oncologists, 19 medical physicists (14 with intermediate titles) and 49 radiotherapists (42 with junior titles, accounting for 86%). The medical teams of 5 hospitals lacked of a 3-level professional title echelon. A total of 67% (33/49) of radiotherapists had ≤ 5 years of working experience. There were 10 medical linear accelerators, 2 sets of after-loading radiotherapy equipment, 8 sets of CT simulation positioning equipment, 1 X-ray simulation positioning machine, and 35 sets of radiotherapy quality control instruments. Conformal intensity-modulated radiotherapy and volumetric arc-modulated radiotherapy could be simultaneously carried out in 8 hospitals. In 2023, 5010 patients were treated with medical linear accelerators and 171 patients were treated with after-loading radiotherapy. Two hospitals carried out scientific research, 1 hospital accepted trainees, and 2 hospitals provided teaching for interns. The results of quality control supervision and inspection showed that the equipment from 8 non-public hospitals could basically meet the needs of radiotherapy and daily equipment quality control.Conclusions:The organizational structure of radiotherapy departments in non-public hospitals in Beijing are generally reasonable, with relatively complete equipment and personnel configurations. However, multiple issues such as an insufficient number of personnel, unbalanced personnel structures, lack of work experience, low professional titles, and inadequate research and teaching capabilities still exist, which require further improvements.

Objective:To explore the clinical phenotype and genetic characteristics of a patient with Progressive pseudorheumatoid dysplasia (PPRD) due to compound heterozygous variants of CCN6 gene. Methods:A patient who was admitted to Qilu Hospital of Shandong University due to " bilateral finger joint deformity, bilateral hip and knee joint movement limitation for 19 years" was selected as the study subject. Clinical data of the patient were retrospectively collected. Peripheral blood samples were collected from the patient and her parents and subjected to whole exome sequencing (WES). Long-read sequencing (LRS) and Sanger sequencing were used to verify the candidate variants. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the pathogenicity of candidate variants was classified. This study was approved by the Medical Ethics Committee of Qilu Hospital of Shandong University (Ethics No.: KYLL-202502 061).Results:The patient, a 23-year-old female, presented with progressive polyarticular deformity, limited movement and abnormal growth and development since childhood. She was initially misdiagnosed as Ankylosing spondylitis and had poor response to sulphasalazine and etoricoxib treatment. WES revealed that she has harbored two heterozygous variants of the CCN6 gene (NM_198239.2), namely c. 348C>A and c. 676G>C. LRS confirmed that the two variants are located on two homologous chromosomes and constitute compound heterozygous variants. Based on the ACMG guidelines, both variants were rated as pathogenic (PVS1+ PM2_Supporting+ PM3; PM1+ PM2_Supporting+ PM3_Supporting+ PM5+ PP3_Strong). The c. 676G>C variant has not been recorded by the HGMD and ClinVar databases. Conclusion:The c. 348C>A and c. 676G>C compound heterozygous variants of the CCN6 gene probably underlay the pathogenesis of PPRD in this patient. Above finding has enriched the mutational spectrum of PPRD and provided a basis for the clinical diagnosis and genetic counseling.

Objective:Primary sclerosing cholangitis (PSC) is a rare autoimmune disease. This study aims to describe the baseline characteristics and clinical outcomes of Chinese PSC patients and explore risk factors associated with prognosis, addressing the lack of long-term prognostic analysis in China.Methods:Clinical data of PSC patients were retrospectively collected from May 2009 to June 2023 in Beijing Friendship Hospital Affiliated to Capital Medical University, and patient follow-up was conducted through outpatient visits, telephone calls, and medical record reviews. The Cox proportional hazards model and the Kaplan-Meier method were employed to identify risk factors and estimate transplant-free survival.Results:A total of 65 PSC patients were enrolled, with male patients accounting for 50.8% and an average age of onset of 44 years. The disease types primarily included large duct PSC (57.9%) and whole duct PSC (22.8%). Most patients (78.5%) sought medical attention due to symptoms, with common clinical manifestations including jaundice (32.3%), fatigue (23.1%), abdominal discomfort (21.5%), pruritus (16.9%), and fever (10.8%). A total of 19 patients (29.2%) had concomitant ulcerative colitis. Compared to large duct PSC or whole duct PSC, small duct PSC showed a lower proportion of concomitant ulcerative colitis ( P<0.001) and milder baseline disease severity. After a median follow-up of 29 months (interquartile range: 11,53), 19 patients experienced liver transplantations and/or liver disease-related deaths. The overall 2-year and 5-year transplant-free survival rates for PSC patients were 76.0% and 59.5%, respectively. Elevated bile acid levels were identified as an independent risk factor for poor outcomes in PSC patients. Conclusion:The study population of Chinese PSC patients predominantly consisted of middle-aged males, characterized by a low ratio of asymptomatic cases, a low incidence of associated inflammatory bowel disease, and a low rate of transplant-free survival. Elevated bile acid level was identified as an independent risk factor for poor outcomes in PSC patients.

Objective:To explore the distribution characteristics of HBsAg levels in treatment-na?ve and treatment-experienced patients with chronic hepatitis B (CHB) in China.Methods:Data were obtained from the China Registry of Hepatitis B (CR-HepB) platform from the establishment of the platform to April 11, 2024. Patients with CHB who were treatment-na?ve and treatment-experienced with nucleos(t)ide analogs (NAs) were included. Relevant clinical data were collected. The distribution of hepatitis B surface antigen (HBsAg) status, as well as the levels in populations of different age groups after different antiviral treatment durations, were retrospectively analyzed. Normally and non-normally distributed measured data were represented by Mean± SD, and M( Q1, Q3). Results:A total of 13 505 treatment-na?ve patients and 6 390 treatment-experienced patients were included in the analysis. The proportions of treatment-na?ve patients with HBsAg<100, <500, and <1 500 IU/mL were 10.51%, 28.47%, and 46.85%, and the corresponding proportions of treatment-experienced patients were 12.88%, 29.84%, and 52.07%. The proportions of treatment-na?ve patients with HBsAg levels≥1 500, ≥3 000, and≥8 000 IU/mL were 53.15%, 38.17%, and 15.62%, and the corresponding proportions of treatment-experienced patients were 47.93%, 31.77%, and 10.39%. HBsAg level showed a trend of gradual decrease with the increase of antiviral treatment time. The proportion of treatment-experienced patients with HBsAg<100 IU/mL increased from 12.73% when the treatment duration was less than three years to 26.92% when the treatment duration was≥10 years, while the proportion of patients with HBsAg levels≥3 000 IU/mL or≥8 000 IU/mL decreased from 34.66% to 23.08% and from 12.19% to 5.77%, respectively. The proportion of patients with HBsAg<100, <500, and<1 500 IU/mL increased with age, while the proportion of patients with HBsAg≥1 500, ≥3 000, and ≥8 000 IU/mL decreased sequentially.Conclusions:The CR-HepB platform provides a basis for clarifying the serum HBsAg levels in treatment-na?ve and treatment-experienced CHB patients in China. The HBsAg status indicates that with a prolonged antiviral treatment duration, there is a gradual decline trend in HBsAg level.

China has made remarkable achievements during the past three decades in controlling chronic hepatitis B virus infection. The overall HBsAg positivity rate dropped from 9.72% in 1992 to 5.86% in 2020. Particularly, the HBsAg positivity rate among children under five years old has dropped from 9.67% in 1992 to 0.30% in 2020. This transition is due to the universal vaccination of newborns with hepatitis B vaccine since 1992, which has averted over 40 million of HBV infections and seven million hepatitis B-related deaths. However, there are still around 75 million cases of chronic hepatitis B virus infection in China, of which about 59.78% of the infected individuals were aware of their infection status before survey, and about 30 million people have not yet been diagnosed. Among the confirmed infected individuals, 38.25% (about 17 million people) have indications for antiviral treatment, but only 17.33% (about 3 million people) are receiving antiviral treatment. Therefore, in order to accelerate the actualization of the World Health Organization's goal of eliminating viral hepatitis as a public health threat by 2030, China has taken a series of active measures in recent years, including updating clinical guidelines and expanding treatment indications to improve the coverage of diagnosis and treatment. At the same time, Chinese pharmaceutical companies and academia have made significant progress in the research and development of innovative hepatitis B therapies, laying the foundation for achieving the functional cure of chronic hepatitis B. Furthermore, China has developed a comprehensive management model for the prevention, control, and elimination of hepatitis B through evidence-based public health interventions, optimized clinical management strategies, and promotion of innovative drugs, providing valuable experience for the world.

Objective:To investigate the role of dynamic changes in liver stiffness measurement (LSM) in predicting liver-related end-point events (LREs) occurrence in patients with chronic hepatitis B (CHB) with liver fibrosis during long-term antiviral therapy.Methods:Data were collected from CHB patients whose liver biopsy results showed Metavir fibrosis stage F2～F4 or clinically diagnosed cirrhosis. Entecavir antiviral therapy was mainly administered. Follow-up was conducted once every six months. Clinical data such as demographic information, blood routine tests, liver biochemical parameters, HBV virological and serological test results, and LSM were collected. Dynamic changes in LSM were categorized into four types based on LSM levels before treatment (0y) and following two years of antiviral therapy (2y) : (1) LSM 0y < 10 kPa and LSM 2y < 10 kPa, i.e., LSM persisted < 10 kPa; (2) LSM 0y < 10 kPa and LSM 2y ≥ 10 kPa, i.e., LSM increased to ≥ 10 kPa; (3) LSM 0y ≥ 10 kPa and LSM 2y < 10 kPa, i.e., LSM decreased to < 10 kPa; (4) LSM 0y ≥ 10 kPa and LSM 2y ≥ 10 kPa, i.e., LSM persisted ≥ 10 kPa. The predictive role of the dynamic changes of LSM in the occurrence of LREs was analyzed. The Wilcoxon rank-sum test was used for quantitative data. Fisher's exact test was used for categorical data. Multivariate analysis was performed using the Cox proportional hazards regression model. Survival curves were plotted and compared using the Kaplan-Meier. Results:A total of 713 CHB cases with liver fibrosis were included, among whom 512 had cirrhosis. The cumulative incidence of LREs following two years of antiviral therapy was low in patients with LSM 0y < 10 kPa during follow-up (all patients: LSM persisted < 10 kPa 1.6% vs. LSM increased to ≥ 10 kPa 0%; cirrhosis subgroup: LSM persisted < 10 kPa 0% vs. LSM increased to ≥ 10 kPa 0%). The 5-year cumulative incidence of LREs following two years of antiviral treatment was significantly higher in patients with LSM0y ≥ 10 kPa than in those with LSM persisting ≥ 10 kPa and those with LSM decreasing to < 10 kPa during follow-up (all patients: LSM persisted ≥ 10 kPa 12.4% vs. LSM decreased to < 10 kPa 3.6%; cirrhosis subgroup: LSM persisted ≥ 10 kPa 12.6% vs. LSM decreased to < 10 kPa 4.3%). Patients with LSM persisting at ≥ 10 kPa had a significantly increased risk of LREs following two years of antiviral treatment compared with those whose LSM decreased to <10 kPa during follow-up after adjusting for age, gender, baseline body mass index, platelet count, and alanine aminotransferase (all patients, aHR=2.96, 95% CI: 1.41～6.24, P=0.005; cirrhosis subgroup, aHR=2.74, 95% CI:1.26～5.95, P=0.011). Conclusions:LSM<10 kPa before antiviral treatment had a lower risk of liver-related endpoint events following two years of treatment among CHB patients with liver fibrosis. LSM ≥10 kPa before antiviral treatment and LSM persisted ≥10 kPa two years following treatment had a significantly higher occurrence risk of liver-related endpoints than LSM<10 kPa following treatment among CHB patients with liver fibrosis.

Objective:To dynamically assess liver fibrosis using magnetic resonance elastography (MRE) and explore factors associated with fibrosis reversal in patients with metabolic dysfunction-associated steatohepatitis (MASH).Methods:This study included data from patients diagnosed with MASH by liver biopsy who underwent at least two MRE examinations. Patients were divided into a fibrosis reversal group and a non-reversal group according to whether MRE values decreased by 20% during follow-up. Differences in clinical data between the groups were compared using analysis of variance, the Kruskal-Wallis test, and the chi-square test. Univariate and multivariate logistic regression analyses were used to explore independent risk factors for fibrosis reversal in MASH.Results:A total of 46 cases were included in this study (mean age 50.1±12.3 years, BMI 26.1±3.1 kg/m2). Among them, the reversal group accounted for 26.1%. The rate of decrease in MRI proton density fat fraction (PDFF) was significantly higher in the reversal group (-50.0% vs. -8.1%, P=0.001) than in the non-reversal group between the two MRE examinations. The reversal group showed a more significant change rate of decreases in fasting insulin (-37.3% vs. -3.6%, P=0.011), insulin resistance index (-38.6% vs. -6.5%, P=0.044), and ALP (-24.9% vs. 0, P=0.004). Multivariate logistic regression analysis indicated that the rate of change in MRI PDFF was an independent predictor of fibrosis reversal ( OR=0.96, 95% CI: 0.92-1.00, P=0.046). Conclusion:A decrease in MRI proton density fat fraction levels is independently associated with liver fibrosis reversal in MASH, suggesting that intervention targeting liver fat content may be an effective treatment strategy.

Objective:To compare the influence of definition methods of different isocenter on postoperative volumetric modulated arc therapy (VMAT) plans for patients with high-grade glioma (HGG),and to explore the feasibility of determining the treatment isocenter at location stage of computed tomography (CT) simulation system (CT-Sim). Methods:A total of 30 HGG patients who received radiotherapy after surgery in Peking University International Hospital from September 1,2021 to December 31,2022 were selected. 4 kinds of methods,which included center of mass (CT-COM) of target region of tumor bed was delineated by CT-Sim location stage,geometric center of target region of treatment plan system (TPS-Geo),whole brain geometric center of treatment plan system (TPS-Head) and center of mass of target region of TPS (TPS-COM),were respectively adopted to definite treatment isocenter for each patient,and they were respectively set as CT-COM group,TPS-Geo group,TPS-Head group and TPS-COM group to design 4 groups of VMAT treatment. The parameters included dose,conformity index (CI),uniformity index (HI) and machine unite (MU) among 4 groups of target region and organ at risk (OAR) were compared. Results:The doses of target region and OAR of four groups were similar,and all of them can meet the requirements of clinical dosimetry and clinical OAR dose limitation,without statistically significant differences (P>0.05). The covered volumes that brain tissues received 10,20,30,40 and 50 Gy doses in CT-COM group,TPS-Geo group and TPS-COM group closed to the maximum dose,the maximum dose of 2％ volume and mean dose (Dmax,D2％,Dmean) of brainstem,bilateral lens,optic chiasm,bilateral optic nerve and pituitary gland. The V10,V20,V30 of brain tissue,and Dmax,D2％,and Dmean of brainstem in TPS-Head group were slightly higher than those in other three groups,and the Dmean of lens,and Dmax,D2％ and Dmean of optic nerve at health side of TPS-Head group were slightly lower than those of other three groups,without statistically significant differences (P>0.05). Conclusion:The four VMAT plans of defining the treatment isocenter can meet the clinical dosimetric requirements,and can determine the isocenter at CT-Sim location stage,which can effectively avoid the occurrence of errors in the reduction of radiotherapy,and increase precision of delivery of radiotherapy dose,and enhance effective utilization rate of equipment.