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.Construction and application of a graded nursing program for exercise rehabilitation in children with hematopoietic stem cell transplantation
Yinzhi YAN ; Fang WEN ; Min WANG ; Xuemei ZHOU ; Jinling MA ; Huifang WU ; Wenying YAO
Chinese Journal of Nursing 2025;60(9):1036-1042
Objective To construct a graded nursing program for exercise rehabilitation of children with hematopoietic stem cell transplantation(HSCT)and carry out a preliminary application,aiming to provide a basis for healthcare personnel to guide the exercise rehabilitation of children with HSCT.Methods Literature analysis and Delphi expert correspondence were used to construct a graded nursing program of exercise rehabilitation for children with HSCT.Through the convenience sampling method,children with HSCT in the hematology department of a tertiary-level children's hospital in Suzhou City were selected as the study subjects from June-December 2023,of which 26 children with HSCT from September-December 2023 served as an experimental group,and 23 children with HSCT from June-August 2023 as a control group.The experimental group was applied with the graded nursing program of exercise rehabilitation,and the control group was applied with the conventional nursing care.The comparison of cardiorespiratory fitness and adverse events between the 2 groups after 8 weeks of exercise intervention was conducted,and the adherence to the exercise intervention programme in the experimental group was recorded.Results The final constructed graded care program of exercise rehabilitation for children with HSCT included 6 primary indicators,20 secondary indicators,and 44 tertiary indicators.Finally,21 children in the experimental group and 23 children in the control group were included in the study.After the intervention,the 6 min walking distance of the experimental group increased compared with that of the control group,and the difference was statistically significant(P<0.001).85.71%of children in the experimental group had good exercise compliance,and there was no adverse events in both groups.Conclusion The constructed graded nursing program of exercise rehabilitation for children with HSCT is scientific,reasonable,safe and feasible,which can improve the cardiorespiratory capacity of children with HSCT and provide the basis for their exercise rehabilitation.
3.Effect of rosa roxburghii Tratt polysaccharides on insulin resistance in gestational diabetes mellitus rats by regulating adenosine monophosphate-activated proteinkinase/peroxlsome proliferator-activated receptor-γ coactlvator-1α signaling pathway
Xiuping CHI ; Jinling YAN ; Wenhua CUI ; Ling LIU ; Lixuan WEN
Chinese Journal of Diabetes 2025;33(7):536-542
Objective To investigate the effect of rosa roxburghii Tratt polysaccharides(RRTP)on insulin resistance(IR)in rats with gestational diabetes mellitus(GDM)by regulating adenosine monophosphate-activated protein kinase(AMPK)/peroxlsome proliferator-activated receptor-γ coactlvator-1α(PGC-1α)signaling pathway.Methods Ten pregnant rats served as normal control(NC)group,and 50 GDM rats were randomly divided into GDM group,RRTP 100 mg/kg(RRTP 100)group,RRTP 200 mg/kg(RRTP 200)group,positive drug(PD)group,RRTP 200 mg/kg+Dorsomorphin(RRTP 200+Dorsomorphin)group.The indexes of glucose and lipid metabolism,superoxide dismutase(SOD),malondialdehyde(MDA),interleukin-6(IL-6),tumor necrosis factor-α(TNF-α)and pathological changes were evaluated in each group,and Schmidt scoring shall be performed.Additionally,apoptosis and the expression of proteins related to the AMPK/PGC-1α pathway in tissues shall be detected.Results Fasting plasma glucose,fasting insulin,homeostatic model assessment-IR,total cholesterol,triglycerides,low-density lipoprotein cholesterol,MDA,IL-6,TNF-α,Schmidt score and apoptosis index were higher in GDM group than in NC group(P<0.05),while the expressions of high-density lipoprotein cholesterol,SOD,p-AMPK/AMPK,p-AMPK and PGC-1α protein were lower in GDM group than in NC group(P<0.05).The above indexes improved in RRTP 100,RRTP 200 and PD groups.In the RRTP 200+Dorsomorphin group,the ameliorative effect of RRTP on IR was reversed.Conclusions RRTP improve IR in GDM rats by activating the AMPK/PGC-1α signaling pathway.
4.Chronic hypoxia exacerbates pulmonary hypertension-induced right heart remodeling in mice by inhibiting GLS1-regulated glutaminolysis
Jinling HUANG ; Ranran YAN ; Guodan BAO ; Zhenzhong BAI
Chinese Journal of Pathophysiology 2025;41(11):2130-2136
AIM:This study aims to identify how glutamine metabolism affects pulmonary hypertension-in-duced right heart remodeling under chronic hypoxia.METHODS:C57BL/6J mice were randomly divided into control(n=10)and hypoxia(n=10)groups.The hypoxia group was exposed to a hypobaric chamber simulating an altitude of approxi-mately 5,500 m for 28 days.Right ventricular systolic pressure(RVSP)was measured by right heart catheterization.The right ventricle(RV),left ventricle,and interventricular septum(LV+S)were weighed,and the RV/(LV+S)ratio was cal-culated as an index of right ventricular hypertrophy.Metabolomics analysis using UPLC-Orbitrap mass spectrometry was performed on right ventricular tissues.Hematoxylin and eosin staining and immunofluorescence were used to detect YAP and GLS1 co-localization and expression intensity in the myocardium.Expression levels of Yes-associated protein(YAP),phosphorylated YAP(p-YAP),and glutaminase 1(GLS1)from the Hippo pathway were measured by Western blot in mouse right ventricular tissue.RESULTS:The RVSP and RVHI values,along with myocardial fiber sizes and volumes,were significantly increased in the hypoxia group compared to controls(P<0.01).Glutamine metabolism metabolites in right ventricular tissue were significantly decreased(P<0.01).Immunofluorescence revealed decreased GLS1 and in-creased YAP response intensity in the hypoxia group myocardium(P<0.01).Notably,p-YAP protein and GLS1 expres-sion decreased under hypoxic conditions(P<0.05 or P<0.01,respectively).CONCLUSION:Chronic hypoxia exacer-bates pulmonary hypertension-induced right heart remodeling by inhibiting glutaminolysis through glutaminase 1 via the Hippo pathway in mice.
5.Supramolecular prodrug inspiried by the Rhizoma Coptidis - Fructus Mume herbal pair alleviated inflammatory diseases by inhibiting pyroptosis.
Wenhui QIAN ; Bei ZHANG ; Ming GAO ; Yuting WANG ; Jiachen SHEN ; Dongbing LIANG ; Chao WANG ; Wei WEI ; Xing PAN ; Qiuying YAN ; Dongdong SUN ; Dong ZHU ; Haibo CHENG
Journal of Pharmaceutical Analysis 2025;15(2):101056-101056
Sustained inflammatory responses are closely related to various severe diseases, and inhibiting the excessive activation of inflammasomes and pyroptosis has significant implications for clinical treatment. Natural products have garnered considerable concern for the treatment of inflammation. Huanglian-Wumei decoction (HLWMD) is a classic prescription used for treating inflammatory diseases, but the necessity of their combination and the exact underlying anti-inflammatory mechanism have not yet been elucidated. Inspired by the supramolecular self-assembly strategy and natural drug compatibility theory, we successfully obtained berberine (BBR)-chlorogenic acid (CGA) supramolecular (BCS), which is an herbal pair from HLWMD. Using a series of characterization methods, we confirmed the self-assembly mechanism of BCS. BBR and CGA were self-assembled and stacked into amphiphilic spherical supramolecules in a 2:1 molar ratio, driven by electrostatic interactions, hydrophobic interactions, and π-π stacking; the hydrophilic fragments of CGA were outside, and the hydrophobic fragments of BBR were inside. This stacking pattern significantly improved the anti-inflammatory performance of BCS compared with that of single free molecules. Compared with free molecules, BCS significantly attenuated the release of multiple inflammatory mediators and lipopolysaccharide (LPS)-induced pyroptosis. Its anti-inflammatory mechanism is closely related to the inhibition of intracellular nuclear factor-kappaB (NF-κB) p65 phosphorylation and the noncanonical pyroptosis signalling pathway mediated by caspase-11.
6.Long-term protective efficacy of recombinant CHO cell-derived hepatitis B vaccine:A 26-year follow-up study in Zhengding county
Qiudong SU ; Zhiyong HAO ; Feng QIU ; Lili QIAO ; Huijuan JIA ; Shuang ZHANG ; Pengpeng YUAN ; Juan CHU ; Zhiyong ZHANG ; Junfang YAO ; Jinling WEI ; Yan WANG ; Peiqi ZHAO ; Yujiao LIU ; Zhao GAO ; Jingchen MA ; Feng WANG ; Haisong ZHOU
Chinese Journal of Experimental and Clinical Virology 2025;39(4):434-440
Objective:To evaluate the long-term protective efficacy of the recombinant Chinese hamster ovary cell-derived hepatitis B vaccine(CHO-HepB)26 years post-vaccination in the rural China.Methods:Zhengding county,Hebei province was designated as a rural monitoring site for CHO-HepB efficacy. Study participants included individuals born between 1997 and 1999 who had completed the three-dose CHO-HepB primary series without booster doses. A cross-sectional survey was conducted in late 2024 using random sampling. Demographic and vaccination history data were collected via questionnaires,and hepatitis B virus(HBV)serological markers were detected using chemiluminescence. Historical surveillance data were integrated to infer infection statuses of HBsAg-positive individuals and evaluate longitudinal trends in anti-HBs seropositivity and antibody titers.Results:Among 178 participants(mean time since vaccination:26.2 years),the seroprevalence rates were 0.6% for HBsAg(95% CI:0.0%-1.6%),64.6% for anti-HBs(95% CI:57.6%-71.6%),and 1.1% for anti-HBc(95% CI:0.0%-2.7%). Compared to the pre-vaccination baseline HBsAg positivity of 11.3% in children under 10 years of age,the estimated vaccine protection rate was 95%. Two notable cases were identified:one with concurrent HBsAg and anti-HBc positivity and one with anti-HBs and anti-HBc positivity,suggestive of transient HBV exposure(1999—2009)without chronicity. Natural immune boosting was inferred for the latter case based on anti-HBs titer dynamics. Longitudinal analysis of four prior cross-sectional surveys(2005,2009,2013,and 2017)revealed no significant upward trends in HBsAg and anti-HBc positivity(both P>0.05)over 26 years,while anti-HBs seropositivity declined significantly( P<0.05)from 6 to 26 years post-vaccination. Conclusion:The CHO-HepB vaccine demonstrates sustained immunological persistence and robust long-term protection up to 26 years post-immunization. Continued emphasis on rigorous implementation of mother-to-child transmission prevention strategies is critical for future hepatitis B control.
7.Effect of rosa roxburghii Tratt polysaccharides on insulin resistance in gestational diabetes mellitus rats by regulating adenosine monophosphate-activated proteinkinase/peroxlsome proliferator-activated receptor-γ coactlvator-1α signaling pathway
Xiuping CHI ; Jinling YAN ; Wenhua CUI ; Ling LIU ; Lixuan WEN
Chinese Journal of Diabetes 2025;33(7):536-542
Objective To investigate the effect of rosa roxburghii Tratt polysaccharides(RRTP)on insulin resistance(IR)in rats with gestational diabetes mellitus(GDM)by regulating adenosine monophosphate-activated protein kinase(AMPK)/peroxlsome proliferator-activated receptor-γ coactlvator-1α(PGC-1α)signaling pathway.Methods Ten pregnant rats served as normal control(NC)group,and 50 GDM rats were randomly divided into GDM group,RRTP 100 mg/kg(RRTP 100)group,RRTP 200 mg/kg(RRTP 200)group,positive drug(PD)group,RRTP 200 mg/kg+Dorsomorphin(RRTP 200+Dorsomorphin)group.The indexes of glucose and lipid metabolism,superoxide dismutase(SOD),malondialdehyde(MDA),interleukin-6(IL-6),tumor necrosis factor-α(TNF-α)and pathological changes were evaluated in each group,and Schmidt scoring shall be performed.Additionally,apoptosis and the expression of proteins related to the AMPK/PGC-1α pathway in tissues shall be detected.Results Fasting plasma glucose,fasting insulin,homeostatic model assessment-IR,total cholesterol,triglycerides,low-density lipoprotein cholesterol,MDA,IL-6,TNF-α,Schmidt score and apoptosis index were higher in GDM group than in NC group(P<0.05),while the expressions of high-density lipoprotein cholesterol,SOD,p-AMPK/AMPK,p-AMPK and PGC-1α protein were lower in GDM group than in NC group(P<0.05).The above indexes improved in RRTP 100,RRTP 200 and PD groups.In the RRTP 200+Dorsomorphin group,the ameliorative effect of RRTP on IR was reversed.Conclusions RRTP improve IR in GDM rats by activating the AMPK/PGC-1α signaling pathway.
8.Chronic hypoxia exacerbates pulmonary hypertension-induced right heart remodeling in mice by inhibiting GLS1-regulated glutaminolysis
Jinling HUANG ; Ranran YAN ; Guodan BAO ; Zhenzhong BAI
Chinese Journal of Pathophysiology 2025;41(11):2130-2136
AIM:This study aims to identify how glutamine metabolism affects pulmonary hypertension-in-duced right heart remodeling under chronic hypoxia.METHODS:C57BL/6J mice were randomly divided into control(n=10)and hypoxia(n=10)groups.The hypoxia group was exposed to a hypobaric chamber simulating an altitude of approxi-mately 5,500 m for 28 days.Right ventricular systolic pressure(RVSP)was measured by right heart catheterization.The right ventricle(RV),left ventricle,and interventricular septum(LV+S)were weighed,and the RV/(LV+S)ratio was cal-culated as an index of right ventricular hypertrophy.Metabolomics analysis using UPLC-Orbitrap mass spectrometry was performed on right ventricular tissues.Hematoxylin and eosin staining and immunofluorescence were used to detect YAP and GLS1 co-localization and expression intensity in the myocardium.Expression levels of Yes-associated protein(YAP),phosphorylated YAP(p-YAP),and glutaminase 1(GLS1)from the Hippo pathway were measured by Western blot in mouse right ventricular tissue.RESULTS:The RVSP and RVHI values,along with myocardial fiber sizes and volumes,were significantly increased in the hypoxia group compared to controls(P<0.01).Glutamine metabolism metabolites in right ventricular tissue were significantly decreased(P<0.01).Immunofluorescence revealed decreased GLS1 and in-creased YAP response intensity in the hypoxia group myocardium(P<0.01).Notably,p-YAP protein and GLS1 expres-sion decreased under hypoxic conditions(P<0.05 or P<0.01,respectively).CONCLUSION:Chronic hypoxia exacer-bates pulmonary hypertension-induced right heart remodeling by inhibiting glutaminolysis through glutaminase 1 via the Hippo pathway in mice.
9.Construction and application of a graded nursing program for exercise rehabilitation in children with hematopoietic stem cell transplantation
Yinzhi YAN ; Fang WEN ; Min WANG ; Xuemei ZHOU ; Jinling MA ; Huifang WU ; Wenying YAO
Chinese Journal of Nursing 2025;60(9):1036-1042
Objective To construct a graded nursing program for exercise rehabilitation of children with hematopoietic stem cell transplantation(HSCT)and carry out a preliminary application,aiming to provide a basis for healthcare personnel to guide the exercise rehabilitation of children with HSCT.Methods Literature analysis and Delphi expert correspondence were used to construct a graded nursing program of exercise rehabilitation for children with HSCT.Through the convenience sampling method,children with HSCT in the hematology department of a tertiary-level children's hospital in Suzhou City were selected as the study subjects from June-December 2023,of which 26 children with HSCT from September-December 2023 served as an experimental group,and 23 children with HSCT from June-August 2023 as a control group.The experimental group was applied with the graded nursing program of exercise rehabilitation,and the control group was applied with the conventional nursing care.The comparison of cardiorespiratory fitness and adverse events between the 2 groups after 8 weeks of exercise intervention was conducted,and the adherence to the exercise intervention programme in the experimental group was recorded.Results The final constructed graded care program of exercise rehabilitation for children with HSCT included 6 primary indicators,20 secondary indicators,and 44 tertiary indicators.Finally,21 children in the experimental group and 23 children in the control group were included in the study.After the intervention,the 6 min walking distance of the experimental group increased compared with that of the control group,and the difference was statistically significant(P<0.001).85.71%of children in the experimental group had good exercise compliance,and there was no adverse events in both groups.Conclusion The constructed graded nursing program of exercise rehabilitation for children with HSCT is scientific,reasonable,safe and feasible,which can improve the cardiorespiratory capacity of children with HSCT and provide the basis for their exercise rehabilitation.
10.Long-term protective efficacy of recombinant CHO cell-derived hepatitis B vaccine:A 26-year follow-up study in Zhengding county
Qiudong SU ; Zhiyong HAO ; Feng QIU ; Lili QIAO ; Huijuan JIA ; Shuang ZHANG ; Pengpeng YUAN ; Juan CHU ; Zhiyong ZHANG ; Junfang YAO ; Jinling WEI ; Yan WANG ; Peiqi ZHAO ; Yujiao LIU ; Zhao GAO ; Jingchen MA ; Feng WANG ; Haisong ZHOU
Chinese Journal of Experimental and Clinical Virology 2025;39(4):434-440
Objective:To evaluate the long-term protective efficacy of the recombinant Chinese hamster ovary cell-derived hepatitis B vaccine(CHO-HepB)26 years post-vaccination in the rural China.Methods:Zhengding county,Hebei province was designated as a rural monitoring site for CHO-HepB efficacy. Study participants included individuals born between 1997 and 1999 who had completed the three-dose CHO-HepB primary series without booster doses. A cross-sectional survey was conducted in late 2024 using random sampling. Demographic and vaccination history data were collected via questionnaires,and hepatitis B virus(HBV)serological markers were detected using chemiluminescence. Historical surveillance data were integrated to infer infection statuses of HBsAg-positive individuals and evaluate longitudinal trends in anti-HBs seropositivity and antibody titers.Results:Among 178 participants(mean time since vaccination:26.2 years),the seroprevalence rates were 0.6% for HBsAg(95% CI:0.0%-1.6%),64.6% for anti-HBs(95% CI:57.6%-71.6%),and 1.1% for anti-HBc(95% CI:0.0%-2.7%). Compared to the pre-vaccination baseline HBsAg positivity of 11.3% in children under 10 years of age,the estimated vaccine protection rate was 95%. Two notable cases were identified:one with concurrent HBsAg and anti-HBc positivity and one with anti-HBs and anti-HBc positivity,suggestive of transient HBV exposure(1999—2009)without chronicity. Natural immune boosting was inferred for the latter case based on anti-HBs titer dynamics. Longitudinal analysis of four prior cross-sectional surveys(2005,2009,2013,and 2017)revealed no significant upward trends in HBsAg and anti-HBc positivity(both P>0.05)over 26 years,while anti-HBs seropositivity declined significantly( P<0.05)from 6 to 26 years post-vaccination. Conclusion:The CHO-HepB vaccine demonstrates sustained immunological persistence and robust long-term protection up to 26 years post-immunization. Continued emphasis on rigorous implementation of mother-to-child transmission prevention strategies is critical for future hepatitis B control.

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