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.Schistosoma japonicum cystatin has protective effects against"two-hit"sepsis in mice by regulating the inflammatory microenvironment
Wenjuan DUO ; Yixiang WANG ; Jiaxing WANG ; Xinlong XU ; Linxian LI ; Dongchen YANG ; Qili SHEN ; Lichun YANG ; Xiaojing LIU ; Qiwang JING ; Liang CHU ; Xiaodi YANG
Journal of Southern Medical University 2025;45(1):110-117
Objective To evaluate the protective effect of Schistosoma japonicum cystatin(rSj-Cystatin)in a mouse mode of"two-hit"sepsis.Methods Sixty male C57BL/6 mice randomized equally into sham-operated group,protein group,"two-hit"modeling group,and protein intervention group.In the former two groups,the mice received an intraperitoneal injection of 100 μL PBS followed by exposure of the cecum and then by intraperitoneal injection of 100 μL PBS or 25 μg rSj-Cystatin 30 min later;In the latter two groups,100 μL PBS containing LPS(5 mg/kg)was injected intraperitoneally 24 h before cecal ligation and puncture(CLP),and 100 μL PBS or 25 μg rSj-Cystatin were injected 30 min after CLP.At 12 h after rSj-Cystatin treatment,6 mice from each group were sacrificed for detection of TNF-α,IL-6,IL-10,TGF-β,iNOS and Arg-1 in the serum,spleen,liver,lung and kidney tissues using ELISA,for examinations of liver,lung and kidney pathologies with HE staining,and for analysis of CD3+CD4+CD25+Foxp3+T cell percentage in the spleen using flow cytometry.The remaining mice were observed for general condition and 72-h survival.Results The 72-h survival rates in the 4 groups were 100%,100%,0%and 20%,respectively,showing significant differences between the latter two groups.The mouse models of"two-hit"sepsis exhibited obvious tissue pathologies and significant elevations of TNF-α and IL-6 in both the serum and tissue homogenate,which were significantly ameliorated by rSj-Cystatin treatment.Treatment with rSj-Cystatin also increased IL-10 and TGF-β levels and spleen CD3+CD4+CD25+Foxp3+T cell percentage.The septic mouse models also showed increased iNOS levels in all the detected tissues and a decreased Arg-1 level in the kidney,and these changes were obviously improved by rSj-Cystatin treatment.Conclusion rSj-Cystatin has a protective effect against"two-hit"sepsis in mice by regulating the inflammatory microenvironment.
3.Effects of Yijing Decoction Containing Serum on Iron Overload-induced Oxidative Stress in Human Ovarian Granulosa Cells
Chengcheng LIANG ; Jing YU ; Heng HU ; Xiaoyu WANG ; Jiaxin TONG ; Xiaoli WANG ; Yang LI ; Jijun CHU
Chinese Journal of Information on Traditional Chinese Medicine 2025;32(9):105-112
Objective To explore the effects and mechanism of Yijing Decoction containing serum on iron overload-induced oxidative stress of human ovarian granulosa cells.Methods SD rats were used to prepare medicated serum and blank serum.Iron dextran was used to induce oxidative damage of SVOG cells.The cells were divided into control group,model group,containing serum group,blank serum group,antioxidant group and iron chelating agent group.After corresponding intervention,cell viability was detected by CCK-8 method,estrogen(E2)and progesterone(P)content in supernatant were detected by ELISA,the contents of intracellular ferrous ion(Fe2+),malondialdehyde(MDA),adenosine triphosphate(ATP)and the activities of superoxide dismutase(SOD),glutathione peroxidase(GSH-Px),catalase(CAT)were detected by the kit,ROS content was detected by DCFH-DA fluorescence probe,the mitochondrial membrane potential was detected by JC-1 fluorescence probe,Western blot and RT-qPCR were used to detect the protein and mRNA expression of nuclear factor erythroid 2-related factor 2(Nrf2),transferrin(Tf),transferrin receptor 1(TfR1),ferritin heavy chain 1(FTH1)and ferritin light chain(FTL).Results Compared with the control group,the viability of SVOG cells decreased in the model group,the contents of E2 and P in cell supernatant decreased,the contents of Fe2+,MDA and ROS increased,the content of ATP and activities of SOD,GSH-Px and CAT decreased,the mitochondrial membrane potential decreased,the protein and mRNA expressions of Nrf2 and FTH1 decreased,and the protein and mRNA expressions of Tf,TfR1 and FTL increased,with statistical significance(P<0.05,P<0.01).Compared with the model group,the viability of SVOG cells in containing serum group,antioxidant group and iron chelating agent group increased,the contents of E2 and P in cell supernatant increased,the contents of Fe2+,MDA and ROS decreased,the content of ATP and activities of SOD,GSH-Px and CAT increased,the mitochondrial membrane potential increased,the protein and mRNA expressions of Nrf2 and FTH1 increased,and the protein and mRNA expressions of Tf,TfR1 and FTL decreased(P<0.05,P<0.01).Conclusion Yijing Decoction containing serum may relieve oxidative stress damage induced by iron overload,improve mitochondrial function,and restore granulosa cell function,thereby enhancing ovarian function,potentially through up-regulating Nrf2,FTH1 expression and down-regulating Tf,TfR1 and FTL expressions.
4.Study on pricing of initiative hospice and palliative care services by service unit
Tian-shu CHU ; Yi-fan XU ; Li-mei JING ; Xue-ying LI ; Xiao-yu ZHANG ; Jun-mei DENG
Chinese Journal of Health Policy 2025;18(2):47-52
Objective:To conduct a study on pricing by service unit to address the problems of hospice and palliative care pricing and fee system in China.Methods:Combining theoretical research and empirical evidence,this study organized the pricing mechanism of initiative hospice and palliative care services and established a graded and categorized pricing strategy.Empirical research was conducted based on real-world data from 36 pilot institutions in typical areas.Results:This study developed a comprehensive pricing framework for value-based classification price standard of initiative hospice and palliative care services from the perspective of incentive regulation.We proposed a pricing plan based on service units,with inpatient bed fee ranging from 459 to 606 yuan or 459 to 1 102 yuan,and home visit fee ranging from 89 to 264 yuan.Conclusions and suggestions:This study proposes a pricing scheme based on the technique and service value with a gradient fluctuation by service unit,and forms a set of price standards with high economic and technical feasibility,which can provide scientific evidences for solving the pricing problem of hospice care.In addition,there is still a need to establish a multi-level incentive compensation mechanism to motivate all levels and types of organisations and healthcare provider,and to promote the high-quality and sustainable development of hospice and palliative care.
5.Association of postoperative biliary tract infection with expressions of sCD14-ST,PCT,TLR4 and TLR2 in peripheral blood of malignant obstructive jaundice patients undergoing PTCD
Jiyao YAO ; Yu GONG ; Jing WANG ; Li FENG ; Xu CHU
Chinese Journal of Nosocomiology 2025;35(5):672-676
OBJECTIVE To explore the association of postoperative biliary tract infection with expressions of soluble cluster of differentiation 14 subtype(sCD14-ST),procalcitonin(PCT),Toll-like receptor(TLR)4 and TLR2 in malignant obstructive jaundice(MOJ)patients undergoing percutaneous transhepatic cholangial drainage(PTCD).METHODS A total of 105 MOJ patients who underwent PTCD in Shengjing Hospital Affiliated to China Medical University form Nov.2021 to Nov.2023 were enrolled in the study and were divided into the infection group with 31 cases and the non-infection group with 74 cases according to the status of postoperative biliary tract infection.The etiological characteristics and drug resistance rates of the infection group were statistically analyzed.The levels of peripheral blood sCD14-ST,PCT,TLR4 and TLR2 were compared between the two groups,and the values of sCD14-ST,PCT,TLR4 and TLR2 in diagnosis of postoperative biliary tract infection in the MOJ patients under-going PTCD wee analyzed.RESULTS Totally 53 strains of pathogens were isolated from 31 patients of the infec-tion group,among which gram-negative bacteria were dominant.Escherichia coli and Enterococcus faecalis were respectively the major species of the gram-negative and gram-positive bacteria.The E.coli was mainly resistant to ampicillin and sensitive to imipenem;the E.faecalis was mainly resistant to ciprofloxacin,tetracycline,erythro-mycin and was sensitive to vancomycin.There were significant differences in the levels of sCD14-ST,PCT,TLR4 and TLR2 between the two groups(P<0.05);the sCD14-ST level of the infection group was(41.12±13.14)ng/ml,higher than(29.35±9.17)ng/ml of the non-infection group(t=5.248,P<0.001).The area under the curve(AUC)value of the joint detection of sCD14-ST,PCT,TLR4 and TLR2 was higher than that of the single detec-tion in diagnosis of postoperative biliary tract infection in the MOJ patients undergoing PTCD(P<0.05).CONCLUSIONS The gram-negative bacteria are the major pathogens isolated from the MOJ patients with postop-erative biliary tract infection.Imipenem and vancomycin are recommended for treatment of the E.coli and E.fae-calis infections.The patients show abnormal expressions of sCD14-ST,PCT,TLR4 and TLR2.The joint detec-tion of the four indexes has high diagnostic value.
6.Effects of Bushen Antai Mixture on Maternal and Foetal Interface Angiogenesis in Recurrent Spontaneous Abortion Mice Based on the VEGF/Hippo/YAP Pathway
Zhe LI ; Chengcheng LIANG ; Haijing CHU ; Heng HU ; Jing YU ; Jijun CHU
Chinese Journal of Information on Traditional Chinese Medicine 2025;32(4):107-112
Objective To observe the effects of Bushen Antai Mixture on angiogenesis at the maternal fetal interface in recurrent spontaneous abortion(RSA)mice;To explore its mechanism in treating RSA based on the VEGF/Hippo/YAP pathway.Methods RSA mouse model and normal pregnancy mouse were constructed using Clark classic method.RSA model mice were randomly divided into model group,progesterone group,Bushen Antai Mixture low-,medium-and high-dosage groups,and normal pregnant mice were used as normal group,with 10 mice in each group.Starting from the first day of pregnancy,each medication group was given the corresponding medication by gavage for 15 days.The absorption rate of mouse embryos was calculated,the morphology of decidual tissue were observed by HE staining,the mRNA and protein expression of VEGF and Hippo/YAP pathway-related molecules of decidual tissue were detected by RT-qPCR and Western blot.Results Compared with the normal group,the absorption rate of embryos in the model group significantly increased(P<0.01),the arrangement of decidual tissue cells were disordered,edematous and degenerated,some nuclei disappeared,the vascular density decreased,the mRNA expressions of VEGF,Yes associated protein(YAP),large tumor suppressor factor(LATS)1 and LATS2 in decidual tissue significantly decreased(P<0.01),the protein expression of VEGF decreased(P<0.01),and the protein expressions of p-YAP/YAP and p-LATS/LATS significantly increased(P<0.01).Compared with the model group,the absorption rate of embryos in each dosage group of Bushen Antai Mixture was significantly reduced(P<0.05,P<0.01),the cells in the decidual tissue were arranged in an orderly manner,and edema degeneration was reduced,the mRNA expressions of VEGF,YAP,LATS1 and LATS2 in decidual tissue significantly increased(P<0.05,P<0.01),and the protein expression of VEGF significantly increased(P<0.05,P<0.01),the protein expressions of p-YAP/YAP and p-LATS/LATS decreased(P<0.05,P<0.01).Conclusion Bushen Antai Mixture can promote the expressions of VEGF,YAP,LATS by activating VEGF/Hippo/YAP signaling pathway,promote angiogenesis at the maternal-fetal interface,reduce the pregnancy loss rate,and play a therapeutic effect of RSA.
7.Relationship between non-renin-dependent aldosterone and left ventricular hypertrophy in essential hypertension
Guili CHANG ; Changyuan LIU ; Mingchun LI ; Zhe HU ; Jing CHEN ; Qun'an CAO ; Shaoli CHU ; Xin CHEN
Journal of Shanghai Jiaotong University(Medical Science) 2025;45(10):1372-1377
Objective·To analyze the influencing factors of left ventricular mass index(LVMI)in patients with essential hypertension,and explore the relationship between aldosterone levels and left ventricular hypertrophy(LVH).Methods·A total of 155 patients with essential hypertension,hospitalized in the Hypertension Department of the Northern Campus of Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,from January 2013 to December 2019,and excluded from primary aldosteronism by saline load test(post-saline suppression plasma aldosterone<60 pg/mL),were enrolled.General clinical data(age,gender,smoking status,duration of hypertension,etc.),physical examination data(blood pressure and body mass index),blood biochemistry(renal function,electrolytes,fasting blood glucose,and lipids),urinary sodium,and relevant hormones(basal and activated aldosterone,basal and activated renin,urinary aldosterone,post-saline suppression aldosterone,etc.)were collected.LVMI was evaluated by echocardiography.Pearson correlation analysis was used to assess the linear association between LVMI and each variable.Binary Logistic regression models were applied to screen independent risk factors for LVH.Multiple linear regression models were used to assess the impact of variables on LVMI.Results·The mean age of the 155 patients was(46.85±11.08)years,with 51.6%being male.Pearson correlation analysis showed that LVMI was significantly positively correlated with post-saline suppression aldosterone(r=0.334,P<0.001),age(r=0.184,P=0.032),duration of hypertension(r=0.241,P=0.005),systolic blood pressure(r=0.280,P=0.001),and pulse pressure(r=0.339,P<0.001).No significant correlations were found with diastolic blood pressure,body mass index,fasting blood glucose,total cholesterol,low-density lipoprotein cholesterol,triglyceride,high-density lipoprotein cholesterol,urinary sodium,basal aldosterone,activated aldosterone,or urinary aldosterone.After adjusting for confounders,including gender,smoking history,age,duration of hypertension,body mass index,pulse pressure,systolic blood pressure,fasting blood glucose,and total cholesterol,binary Logistic regression showed that each 1 pg/mL increase in post-saline suppression aldosterone was associated with a 5.1%increased risk of LVH(OR=1.051,95%CI 1.016?1.088,P=0.004).Multiple linear regression identified suppressed aldosterone(β=0.359,P<0.001),duration of hypertension(β=0.168,P=0.046),and pulse pressure(β=0.226,P=0.008)as independent influencing factors for LVMI.Conclusion·Suppressed aldosterone is an independent influencing factor for LVH in patients with essential hypertension.
8.Relationship between non-renin-dependent aldosterone and left ventricular hypertrophy in essential hypertension
Guili CHANG ; Changyuan LIU ; Mingchun LI ; Zhe HU ; Jing CHEN ; Qun'an CAO ; Shaoli CHU ; Xin CHEN
Journal of Shanghai Jiaotong University(Medical Science) 2025;45(10):1372-1377
Objective·To analyze the influencing factors of left ventricular mass index(LVMI)in patients with essential hypertension,and explore the relationship between aldosterone levels and left ventricular hypertrophy(LVH).Methods·A total of 155 patients with essential hypertension,hospitalized in the Hypertension Department of the Northern Campus of Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,from January 2013 to December 2019,and excluded from primary aldosteronism by saline load test(post-saline suppression plasma aldosterone<60 pg/mL),were enrolled.General clinical data(age,gender,smoking status,duration of hypertension,etc.),physical examination data(blood pressure and body mass index),blood biochemistry(renal function,electrolytes,fasting blood glucose,and lipids),urinary sodium,and relevant hormones(basal and activated aldosterone,basal and activated renin,urinary aldosterone,post-saline suppression aldosterone,etc.)were collected.LVMI was evaluated by echocardiography.Pearson correlation analysis was used to assess the linear association between LVMI and each variable.Binary Logistic regression models were applied to screen independent risk factors for LVH.Multiple linear regression models were used to assess the impact of variables on LVMI.Results·The mean age of the 155 patients was(46.85±11.08)years,with 51.6%being male.Pearson correlation analysis showed that LVMI was significantly positively correlated with post-saline suppression aldosterone(r=0.334,P<0.001),age(r=0.184,P=0.032),duration of hypertension(r=0.241,P=0.005),systolic blood pressure(r=0.280,P=0.001),and pulse pressure(r=0.339,P<0.001).No significant correlations were found with diastolic blood pressure,body mass index,fasting blood glucose,total cholesterol,low-density lipoprotein cholesterol,triglyceride,high-density lipoprotein cholesterol,urinary sodium,basal aldosterone,activated aldosterone,or urinary aldosterone.After adjusting for confounders,including gender,smoking history,age,duration of hypertension,body mass index,pulse pressure,systolic blood pressure,fasting blood glucose,and total cholesterol,binary Logistic regression showed that each 1 pg/mL increase in post-saline suppression aldosterone was associated with a 5.1%increased risk of LVH(OR=1.051,95%CI 1.016?1.088,P=0.004).Multiple linear regression identified suppressed aldosterone(β=0.359,P<0.001),duration of hypertension(β=0.168,P=0.046),and pulse pressure(β=0.226,P=0.008)as independent influencing factors for LVMI.Conclusion·Suppressed aldosterone is an independent influencing factor for LVH in patients with essential hypertension.
9.Study on the construction of evaluation index system for service capacity of integrated Traditional Chinese and Western medicine trauma rescue:Based on Delphi method and analytic hierarchy process
Jing LI ; Chi ZHOU ; Chu-xi LIANG ; Lei YANG
Chinese Journal of Health Policy 2025;18(9):23-30
Objective:To construct a scientific and reasonable evaluation index system for the service capacity of integrated Traditional Chinese and Western medicine trauma rescue,providing a tool for assessing the synergistic efficacy of regional trauma rescue systems.Methods:Based on the collaborative governance theory framework,an initial indicator pool was developed through literature analysis and focus group discussions.Two rounds of Delphi expert consultation were conducted to screen indicators,and the analytic hierarchy process was used to determine weights.Results:The final system included 5 first-level indicators,19 second-level indicators,and 45 third-level indicators.Experts positive coefficients(0.923),authority coefficient(0.929)and coordination coefficient(0.24)met requirements.Conclusions:The index system demonstrates scientific rigor and operability,supporting the standardized construction and dynamic optimization of integrated Traditional Chinese and Western medicine trauma rescue services.
10.Assessment of Genotoxicity of Tissue Engineered Materials Based on Improved in vivo Hepatocyte Unscheduled DNA Synthesis(UDS)Assay
Luan-luan WANG ; Li HOU ; Xiang-yu CHU ; Zi-yi YANG ; Ling-xiao SUN ; Xiao-fei WANG ; Qiu-jin QU ; Jing XU ; Zeng-xiang LIU ; Xiao-xia SUN
Progress in Modern Biomedicine 2025;25(17):2740-2748
Objective:An in vivo mammalian hepatocyte Unscheduled DNA Synthesis(UDS)test was used to evaluate the genotoxicity of Cross-linked Sodium Hyaluronate Gel and Bone Repair Materials,providing experimental evidence for establishing a UDS testing method for medical devices and materials.Methods:0.9%sodium chloride injection and cottonseed oil were used as the solvent for test materials and negative control,respectively.N-dimethylnitrosamine(NDMA)was used as the positive control for the early sampling times,and 2-acetylaminofluorene(2-AAF)was used as the positive control for the late sampling times.SD rats were administered a single dose for toxic exposure,and liver tissues were collected at 4 h and 16 h,respectively.Hepatocytes were isolated using collagenase perfusion.After labeling with 5-ethynyl-2'-deoxyuridine(EdU),and the net average fluorescence intensity(NAFI)of cell nuclei and nucleoplasm was measured by fluorescence microscope.Data from 50 cells were used to analyze the DNA repair level.Results:Compared with the negative control groups,the positive control groups(NDMA and 2-AAF)showed highly statistically significant differences in NAFI(P<0.01),indicating successful induction of DNA damage.There was no statistically significant differences between the cross-linked sodium hyaluronate gel groups,bone repair material groups and the negative control group(P>0.05),suggesting that these materials did not significantly induce DNA damage under the experimental conditions.Conclusion:This study first applied EdU labeling technology to the in vivo hepatic UDS assay,achieving non-radioactive labeling through click chemistry reactions.Under the conditions of this study,cross-linked sodium hyaluronate gel and bone repair materials did not exhibit genotoxicity.In the follow-up,the sample range can be expanded and the observation period can be prolonged to further improve the genotoxicity evaluation system of medical devices.

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