1.A Case of Multidisciplinary Treatment for a Patient with Gorham-Stout Disease
Jing HU ; Ying JIN ; Yan ZHANG ; Ji LI ; Wenhui WANG ; Yue CHI ; Chunxu LI ; Zhenjie ZHANG ; Yaping LIU ; Xiaotian CHU ; Jin XU ; Min SHEN
JOURNAL OF RARE DISEASES 2026;5(1):52-59
Gorham-Stout disease(GSD) is a rare osteolytic disorder characterized by spontaneous and progressive osteolysis, along with abnormal angiogenesis and lymphangiogenesis, with no new bone formation. We present a case of a 15-year-old female admitted due to " recurrent right leg pain for 5 years, 11 months after undergoing right femoral fracture surgery". Through comprehensive integration of the patient's clinical phenotype, laboratory tests, imaging findings, pathological examinations, and molecular biological test results, GSD was considered highly likely. A multidisciplinary treatment approach was conducted, including a combination of zoledronic acid and sirolimus to inhibit osteolysis, along with rehabilitation training and orthopedic intervention, providing a personalized and comprehensive treatment strategy.
2.Current status and influencing factors of knowledge-attitude-practice in myopia prevention and control among children and adolescents in Ningbo
Jue WANG ; Xiaotian LIU ; Xia JIN ; Yanli ZHANG ; Hongjun LI ; Honger SUN ; Aiai CHEN ; Yuan TANG
International Eye Science 2026;26(3):518-522
AIM:To investigate the current status and influencing factors of knowledge-attitude-practice in myopia prevention and control among children and adolescents in Ningbo City, thereby providing a scientific basis for formulating targeted prevention strategies.METHODS: Children and adolescents aged 6-12 years old were selected from the medical-school collaborative myopia prevention network in Ningbo City between August 2024 and May 2025 using stratified cluster sampling. Information on myopia prevention knowledge(15 items)and practice(9 items)was collected through questionnaire surveys. Logistic regression models were used to analyze factors influencing myopia occurrence in children and adolescents.RESULTS: A total of 664 children and adolescents aged 6-12 years were enrolled in this study. Participants were divided by age into three groups: 6-7 years old(n=221), 8-9 years old(n=221), and 10-12 years old(n=222). Of the 664 questionnaires distributed, 637 valid questionnaires were returned(201 from the 6-7 age group, 235 from the 8-9 age group, and 201 from the 10-12 age group), yielding an effective response rate of 95.9%. Based on myopia screening results, the non-myopic group comprised 203 participants(31.9%), including 100 males and 103 females, with a mean age of 8.82±1.98 years old. The myopic group comprised 434 participants(68.1%), including 213 males and 221 females, with a mean age of 9.10±1.95 years old. The myopia prevalence rates in the 6-7, 8-9, and 10-12 age groups were 37.8%(76/201), 71.9%(169/235), and 94.0%(189/201), respectively(P<0.001). Regarding the knowledge and practice of myopia prevention, the overall awareness rate in the non-myopic group(59.7%±9.7%)was significantly higher than that in the myopic group(48.7%±8.5%; P<0.001). Additionally, the non-myopic group scored higher on the key practice of “regular eye examinations”(4.27±0.96)compared to the myopic group(4.10±1.05; P<0.05). Logistic regression analysis indicated that age was the primary risk factor for myopia occurrence.CONCLUSION: Age is the dominant factor in the onset of myopia, and there is a phenomenon of “knowledge-practice gap”; the traditional health education model has limitations, and a precise prevention and control system based on developmental patterns should be established.
3.Frailty trajectory and risk factors in elderly hemodialysis patients after SARS-CoV-2 infection
Yifan YANG ; Huayu YANG ; Zongli DIAO ; Xu LIU ; Lan YAO ; Liyan WANG ; Xiaotian SHI ; Xu LI ; Qing MA
Chinese Journal of Geriatrics 2025;44(2):167-172
Objective:To investigate the trajectory of frailty in elderly patients on maintenance hemodialysis(MHD)following SARS-CoV-2 infection and its associated risk factors.Methods:This prospective cohort study focused on elderly patients who underwent baseline frailty assessment(T0)during hemodialysis treatment at Beijing Friendship Hospital for over 3 months between December 1st, 2022, and December 31th, 2022, and were diagnosed with SARS-CoV-2 infection.The Fried Frailty Phenotype was evaluated at 1 month(T1), 3 months(T2), and 6 months(T3)post-infection.Frailty trajectory after infection was analyzed using repeated measurement ANOVA.Patients were divided into stable/improvement or exacerbation groups based on their frailty status at T0 and T3, with logistic regression analysis employed to identify risk factors for different frailty trajectories.Results:A total of 130 elderly maintenance hemodialysis patients, with a median age of 66 years(range: 63-71 years)and 62 males(47.7%), were included in the study.Six months after the infection, a majority of surviving patients saw their frailty scores return to baseline levels.Specifically, 72 patients(55.4%)either maintained or improved to robust or pre-frail states, while 9 patients(6.9%)progressed to a pre-frail state, 18 patients(13.8%)progressed to a frail state, and 31 patients(23.8%)remained in a frail state.Results from multivariate logistic regression analysis indicated that low grip strength( OR: 6.30, 95% CI: 1.48-26.73)and all-cause hospitalization( OR: 5.01, 95% CI: 1.19-21.03)were identified as risk factors for non-frail patients transitioning to frailty( P<0.05). Conclusions:The majority of elderly maintenance hemodialysis patients who survived SARS-CoV-2 infection returned to their baseline level of frailty or showed improvement within 6 months.Non-frail patients with low grip strength or those who were hospitalized were more likely to deteriorate towards frailty.
4.Network analysis of the relationship between self-management level and supportive care demand in maintenance hemodialysis patients
Xue LI ; Hongyi LI ; Yuying FAN ; Jingshu LI ; Xiaotian ZHANG ; Shengnan KUAI
Chinese Journal of Practical Nursing 2025;41(17):1323-1329
Objective:To clarify the complex network structure between self-management levels and supportive care needs of patients undergoing maintenance hemodialysis, utilizing network analysis methods to identify the core and bridging nodes among the variables, thereby defining targets for nursing interventions to implement more precise care strategies.Methods:A cross-sectional survey was conducted from May to November 2023 among 302 outpatient maintenance hemodialysis patients at the hemodialysis centers of two healthcare institutions in Harbin (the Second Affiliated Hospital of Harbin Medical University and Heilongjiang Changjiang Nephrology Specialist Hospital). This involved the use of general information questionnaires, the Hemodialysis Patient Self-Management Scale, and the Supportive Care Needs Scale for Hemodialysis Patients. R language was employed for the network analysis.Results:A total of 300 valid questionnaires were collected, including 186 males and 114 females, with an age range of 23 to 88 years (mean age 55.00 ± 13.78 years). The scores for the dimensions of self-management among dialysis patients were as follows: problem-solving (3.38 ± 0.63), execution of self-care (3.16 ± 0.52), partnership (2.56 ± 0.69), and emotional processing (1.89 ± 0.63). The scores for the dimensions of supportive care needs among dialysis patients were: physiological needs (2.82 ± 1.08), psychological needs (1.51 ± 1.02), social needs (1.97 ± 1.07), emotional needs (1.67 ± 1.12), spiritual needs (2.22 ± 0.77), informational needs (2.83 ± 1.08), and practical needs (2.82 ± 1.03). In network analysis, the strongest intensity was found in the execution of self-care (1.753), and the highest closeness was in psychological needs (0.017). The top three dimensions ranked by bridge strength were social needs (1.463), partnership (1.462), and execution of self-care (1.384). The root mean square error was lowest for psychological needs (0.518) and emotional needs (0.538). The stability and accuracy of the network structure were found to be good.Conclusions:The key intervention targets for nursing care in maintenance hemodialysis patients were identified as executing self-care, psychological needs, emotional needs, and social needs. Among these, executing self-care served as the core intervention focus, while psychological and emotional needs had dominant influences, and social needs exhibited the strongest bridging role. Nursing staff should prioritize these key targets and tailor personalized comprehensive nursing intervention plans to enhance patients′self-management levels and fully meet their care needs.
5.Frailty trajectory and risk factors in elderly hemodialysis patients after SARS-CoV-2 infection
Yifan YANG ; Huayu YANG ; Zongli DIAO ; Xu LIU ; Lan YAO ; Liyan WANG ; Xiaotian SHI ; Xu LI ; Qing MA
Chinese Journal of Geriatrics 2025;44(2):167-172
Objective:To investigate the trajectory of frailty in elderly patients on maintenance hemodialysis(MHD)following SARS-CoV-2 infection and its associated risk factors.Methods:This prospective cohort study focused on elderly patients who underwent baseline frailty assessment(T0)during hemodialysis treatment at Beijing Friendship Hospital for over 3 months between December 1st, 2022, and December 31th, 2022, and were diagnosed with SARS-CoV-2 infection.The Fried Frailty Phenotype was evaluated at 1 month(T1), 3 months(T2), and 6 months(T3)post-infection.Frailty trajectory after infection was analyzed using repeated measurement ANOVA.Patients were divided into stable/improvement or exacerbation groups based on their frailty status at T0 and T3, with logistic regression analysis employed to identify risk factors for different frailty trajectories.Results:A total of 130 elderly maintenance hemodialysis patients, with a median age of 66 years(range: 63-71 years)and 62 males(47.7%), were included in the study.Six months after the infection, a majority of surviving patients saw their frailty scores return to baseline levels.Specifically, 72 patients(55.4%)either maintained or improved to robust or pre-frail states, while 9 patients(6.9%)progressed to a pre-frail state, 18 patients(13.8%)progressed to a frail state, and 31 patients(23.8%)remained in a frail state.Results from multivariate logistic regression analysis indicated that low grip strength( OR: 6.30, 95% CI: 1.48-26.73)and all-cause hospitalization( OR: 5.01, 95% CI: 1.19-21.03)were identified as risk factors for non-frail patients transitioning to frailty( P<0.05). Conclusions:The majority of elderly maintenance hemodialysis patients who survived SARS-CoV-2 infection returned to their baseline level of frailty or showed improvement within 6 months.Non-frail patients with low grip strength or those who were hospitalized were more likely to deteriorate towards frailty.
6.Network analysis of the relationship between self-management level and supportive care demand in maintenance hemodialysis patients
Xue LI ; Hongyi LI ; Yuying FAN ; Jingshu LI ; Xiaotian ZHANG ; Shengnan KUAI
Chinese Journal of Practical Nursing 2025;41(17):1323-1329
Objective:To clarify the complex network structure between self-management levels and supportive care needs of patients undergoing maintenance hemodialysis, utilizing network analysis methods to identify the core and bridging nodes among the variables, thereby defining targets for nursing interventions to implement more precise care strategies.Methods:A cross-sectional survey was conducted from May to November 2023 among 302 outpatient maintenance hemodialysis patients at the hemodialysis centers of two healthcare institutions in Harbin (the Second Affiliated Hospital of Harbin Medical University and Heilongjiang Changjiang Nephrology Specialist Hospital). This involved the use of general information questionnaires, the Hemodialysis Patient Self-Management Scale, and the Supportive Care Needs Scale for Hemodialysis Patients. R language was employed for the network analysis.Results:A total of 300 valid questionnaires were collected, including 186 males and 114 females, with an age range of 23 to 88 years (mean age 55.00 ± 13.78 years). The scores for the dimensions of self-management among dialysis patients were as follows: problem-solving (3.38 ± 0.63), execution of self-care (3.16 ± 0.52), partnership (2.56 ± 0.69), and emotional processing (1.89 ± 0.63). The scores for the dimensions of supportive care needs among dialysis patients were: physiological needs (2.82 ± 1.08), psychological needs (1.51 ± 1.02), social needs (1.97 ± 1.07), emotional needs (1.67 ± 1.12), spiritual needs (2.22 ± 0.77), informational needs (2.83 ± 1.08), and practical needs (2.82 ± 1.03). In network analysis, the strongest intensity was found in the execution of self-care (1.753), and the highest closeness was in psychological needs (0.017). The top three dimensions ranked by bridge strength were social needs (1.463), partnership (1.462), and execution of self-care (1.384). The root mean square error was lowest for psychological needs (0.518) and emotional needs (0.538). The stability and accuracy of the network structure were found to be good.Conclusions:The key intervention targets for nursing care in maintenance hemodialysis patients were identified as executing self-care, psychological needs, emotional needs, and social needs. Among these, executing self-care served as the core intervention focus, while psychological and emotional needs had dominant influences, and social needs exhibited the strongest bridging role. Nursing staff should prioritize these key targets and tailor personalized comprehensive nursing intervention plans to enhance patients′self-management levels and fully meet their care needs.
7.Effects of 1,25-dihydroxyvitamin D 3 and Lactobacillus plantarum on metabolic dysfunction-associated fatty liver disease by regulating intestinal flora
Hongqia HUANG ; Na LIU ; Xiaowei ZHANG ; Lu REN ; Xiaotian LI
Chinese Journal of Clinical Nutrition 2025;33(3):191-199
Objective:To investigate the impacts of 1,25-dihydroxyvitamin D 3 (VD) and Lactobacillus plantarum (LP) on intestinal flora in animals with metabolic dysfunction-associated fatty liver disease (MAFLD) and their possible mechanisms. Methods:A methionine- choline-deficient (MCD) diet-induced rat model of MAFLD was created. Forty 10-week-old male Sprague-Dawley rats were randomized into methionine-choline-sufficient (MCS) group, MCD group, VD group, LP group, and VD-LP group, with 8 rats in each group. The intervention groups (VD group, LP group, and VD-LP group) were gastrically fed with VD peanut oil solution (6 ng/100 g daily), Lactobacillus plantarum solution (2×10 9CFU/100 g daily), and a combination of these two solutions, respectively. MCS group, MCD group, and LP group were given the same volume of peanut oil on a daily basis. Fecal samples from rats were collected at week 4 and analyzed using 16S rRNA gene sequencing for fecal flora structure. Portal vein blood samples were collected to detect liver biochemistry and lipopolysaccharide (LPS) levels. Pathological changes in liver and terminal ileum tissues were observed using hematoxylin and eosin (H&E) staining. Results:Compared with the MCS group, the MCD group exhibited massive steatosis and lipid infiltration in liver tissues, markedly thinned ileum mucosa, severely damaged villi structure, excessive necrotic and shedding of epithelial cells, and infiltration of inflammatory cells. Compared with the histopathological changes in the MCD group, the steatosis and lipid infiltration of liver tissue, the damage to the ileal mucosa structure and epithelial cells, and the infiltration of inflammatory cells were alleviated in the VD group, LP group, and VD-LP group. Compared with MCS group, the MCD group had significantly higher serum alanine aminotransferase (ALT) (158.50±14.03 U/L vs. 20.38±7.39 U/L), aspartate aminotransferase (AST) (43.88±11.36 U/L vs. 25.75±5.90 U/L), total bile acid (TBA) (140.60±11.77 μmol/L vs. 19.96±4.31 μmol/L), and LPS (16.57±1.19 pg/ml vs. 7.43±0.95 pg/ml) (All P<0.001),which confirmed the successful establishment of rat models of MAFLD. Serum ALT, AST, TBA, and LPS levels in all the three intervention groups were significantly lower than those in MCD group, and the most significant reductions in ALT (51.38±9.05 U/L vs. 158.50±14.03 U/L), AST (55.88±12.19 U/L vs. 143.88±11.36 U/L), TBA (21.00±8.17 μmol/L vs. 140.60±11.77 μmol/L), and LPS (9.72±0.71 pg/ml vs. 16.57±1.19 pg/ml) were seen in the VD-LP group. The microbiota in the MCD group predominantly featured Muribaculaceae, while the MCS group and other intervention groups primarily harbored Lactobacillus. Firmicutes and Lactobacillus were significantly decreased in the MCD group ,while Bacteroidete, Proteobacteria, Verrucomicrobiota, Prevotellaceae UCG-003, Escherichia coli, Bacteroides, and Enterococcus increased significantly. The opposite was true for each intervention group. There were significant differences in Lactobacillus between MCD group and the other four groups. Conclusion:VD and LP can remarkably improve lipid deposition in MAFLD by regulating intestinal flora.
8.Current Status, Challenges, and Prospects of Clinical Research on Rare Tumors in China
Sai GE ; Wenfei LI ; Qi WANG ; Jian LI ; Xiaotian ZHANG ; Lin SHEN
JOURNAL OF RARE DISEASES 2025;4(4):399-405
Although the incidence of each individual type of rare tumor is relatively low, their cumulative impact affects a vast patient population, and their survival prognosis is significantly worse than that of common tumors. In recent years, China has made remarkable progress in clinical research on rare tumors, with a rapid increase in the number of studies and a diversified exploration of treatment modalities. However, clinical research on rare tumors still faces numerous challenges, such as uneven distribution of medical resources, the absence of a foundational registration system, and insufficient motivation for original research and development. Looking ahead, key measures including specialized development, a national collaborative group model, full utilization of real-world data, application of novel clinical research designs, and artificial intelligence technologies will strongly drive comprehensive advancements in China′s rare tumor prevention and treatment system.
9.Research Progress in the Diagnosis and Treatment of Pancreatic Acinar Cell Carcinoma
Wenfei LI ; Yuan XIE ; Liyang MO ; Junjie DANG ; Qi WANG ; Yang YANG ; Qiuying SUN ; Zhenping WEN ; Sai GE ; Xiaotian ZHANG
JOURNAL OF RARE DISEASES 2025;4(4):437-445
Pancreatic acinar cell carcinoma (PACC) is a rare exocrine tumor of the pancreas with distinct clinical and pathological features. In recent years, advancements in molecular biology techniques have led to a deeper understanding of the molecular mechanisms underlying PACC. Progress in imaging, endoscopic, and molecular diagnostic technologies has improved the early detection rate of PACC. The primary treatment modalities for PACC include surgical resection, chemotherapy, targeted therapy and immunotherapy; however, the therapeutic efficacy still requires further improvement. This article reviews the current research status of PACC, covering its epidemiology, pathological characteristics, molecular alterations, diagnostic methods, and treatment strategies, and discusses the controversies and future directions in PACC research.
10.Trends in incidence and disease burden of hypertensive disorders in pregnancy in China from 1990 to 2021: an analysis based on Global Burden of Disease data
Manman CHEN ; Wanzhou WANG ; Qu LU ; Jiahui WANG ; Yuankai ZHAO ; Yu JIANG ; Xiaotian LI ; Qiongjie ZHOU
Chinese Journal of Obstetrics and Gynecology 2025;60(9):723-731
Objective:To analyze the changing trends and international differences in the incidence and disability-adjusted life years (DALY) of hypertensive disorders in pregnancy (HDP) in China from 1990 to 2021 based on the Global Burden of Disease (GBD) database.Methods:The data were derived from the GBD database, covering the incidence and DALY data of HDP in countries or regions around the world. Descriptive statistics were used to compare the differences in disease burden between China and different regions of Europe, America and other developed countries, and to analyze the disease burden characteristics of women of childbearing age in different age groups (every 5 years). The Joinpoint regression model was used to identify trend change points. The age-period-cohort model was used to analyze the independent effects of age, period and birth cohort.Results:(1) From 1990 to 2021, the incidence of HDP in China showed an overall downward trend, from 387.18/100 000 in 1990 to 216.35/100 000 in 2021. Compared with the global incidence rate (1990: 1 167.28/100 000; 2021: 923.48/100 000), the incidence rate of HDP in China was relatively low, but it was still higher than that in some developed countries. (2) The analysis of the Joinpoint regression model showed that the incidence rate of HDP in different age groups first decreased, then increased, and finally leveled off. The connection point with the lowest point was in 2005. DALY showed a continuous downward trend in all age groups. Among them, the decline in maternal DALY of pregnant women in all age groups from 15 to 39 years old was more obvious. (3) The age-period-cohort effect model showed that the incidence rate and DALY of HDP in women of childbearing age aged 20 to 24 years reached their peak.Conclusions:The burden of HDP in China continues to decrease, and the disease prevention and control has achieved remarkable results, but the incidence rate is still higher than that in some developed countries. It is suggested that personalized interventions should be developed for different risk groups to further reduce the burden of disease.

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