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.Effect of storage conditions on long-term preservation of PRP growth factors
Qing QI ; Zhaojie LI ; Qiong WU ; Pingping MAO ; Yangzi SUN ; Jianfeng LUAN ; Shujun WANG
Chinese Journal of Blood Transfusion 2025;38(6):759-765
Objective: To compare the changes in the concentration of relevant growth factors released from platelet-rich plasma (PRP) stored at -80℃ by cryopreservation and at 4℃ by refrigerated lyophilization over 2 years, aiming to provide a theoretical basis for prolonging PRP storage duration. Methods: PRP (n=15) was separated using a blood cell separator and stored under -80℃ cryopreservation (F-PRP group) and 4℃ refrigerated freeze-drying conditions (FD-PRP group). The contents of growth factors (PDGF-AA, PDGF-BB, EGF, TGF-β1, and VEGF) in both groups were measured by ELISA at 1, 3, 6, 9, 12 and 24 months. Results: PDGF-AA and VEGF maintained good stability in both groups for up to 24 months. PDGF-BB and TGF-β1 showed high stability in the first 12 months but their stability decreased gradually from 12th to 24th months. EGF demonstrated good stability in the first 6 months, and its stability gradually decreased from the 9th to 24th months. Comparing the F-PRP and FD-PRP groups, the concentrations of the five growth factors in the FD-PRP group were either not statistically different or higher than those in the F-PRP group at all time points. Specifically, the concentrations of EGF were significantly higher in the FD-PRP group at all time points. Conclusion: Both -80℃ freezing and 4℃ freeze-drying enable long-term preservation of PRP. Freeze-drying imposes less stringent storage requirements and facilitates growth factor compared to frozen storage.
3.Establishment and evaluation of an animal model of heart failure with preserved ejection fraction integrating disease and syndrome based on the "deficiency-blood stasis-toxin" pathogenesis
Xiaoqi WEI ; Xinyi FAN ; Feng JIANG ; Wangjing CHAI ; Jinling XIAO ; Fanghe LI ; Kuo GAO ; Xue YU ; Wei WANG ; Shuzhen GUO
Journal of Beijing University of Traditional Chinese Medicine 2025;48(4):501-515
Objective:
This study aimed to construct an animal model of heart failure with preserved ejection fraction (HFpEF) that integrates disease and syndrome based on the "deficiency-blood stasis-toxin" pathogenesis and to evaluate it comprehensively.
Methods:
The HFpEF mouse model was constructed using a combination of Nω-nitro-L-arginine methyl ester (L-NAME) and a high-fat diet. According to the random number table method, SPF-grade male C57BL/6J mice were randomly assigned to the control, L-NAME, high-fat diet, and model groups, 10 in each group. Comprehensive observations and data collection on macroscopic signs (e.g., fur condition, mental state, stool and urine, oral and nasal condition, paw and body condition, etc.) and cardiac function were performed after 10 and 16 weeks of model induction. Additionally, the syndrome evolution was elucidated based on diagnostic criteria for clinical syndromes of heart failure. Furthermore, pathological and molecular biological examinations of myocardial tissue were performed to assess the stability and reliability of the model.
Results:
Mice in the model group showed typical characteristics of syndrome of qi deficiency and blood stasis, as well as syndrome of internal heat accumulation, including lethargy, slow response, dull paw color and oral/nasal color, exercise intolerance, abnormal platelet activation, dry feces, and dark yellow urine. The time window for these syndromes was between 10 and 16 weeks post-modeling. Cardiac function assessments revealed severe diastolic dysfunction, concentric myocardial hypertrophy, and myocardial fibrosis in the model group. Pathological examinations showed a significantly increased collagen deposition in the myocardial interstitium, enlarged cross-sectional area of cardiomyocytes, and sparse coronary microvasculature in the model group. Molecular biological analyses indicated marked activation of the inducible nitric oxide synthase/nuclear factor kappa-light-chain-enhancer of activated B cells/NOD-like receptor family pyrin domain containing 3 inflammatory pathway and significantly elevated inflammation levels in the myocardial tissue of the model group. Although mice in the L-NAME and high-fat diet groups also showed certain manifestations of qi deficiency syndrome, the substantial cardiac damage was relatively limited compared to the control group.
Conclusion
This study has constructed an animal model of HFpEF that integrates disease and syndrome based on the "deficiency-blood stasis-toxin" pathogenesis. The macroscopic and microscopic characteristics of this model are consistent with the manifestations of syndrome of qi deficiency and blood stasis, toxin syndrome, and syndrome of internal heat accumulation. Moreover, it can stably simulate the HFpEF state and reflect phenotypic changes in human disease. This model provides a suitable experimental platform to explore the pathogenesis of HFpEF, evaluate the effectiveness of traditional Chinese medicine (TCM) treatment regimens, and promote in-depth research on TCM syndromes of heart failure.
4.Construction of a combined disease-syndrome animal model of dilated cardiomyopathy with heart failure toxin syndrome and study on potential biomarkers
Feng JIANG ; Jiayang TANG ; Xiangyi QIAN ; Hai PAN ; Aolong HE ; Xiaoqi WEI ; Jinling XIAO ; Wei WANG ; Shuzhen GUO
Journal of Beijing University of Traditional Chinese Medicine 2025;48(5):613-624
Objective To construct an animal model of dilated cardiomyopathy(DCM)with heart failure toxin syndrome that conforms to the characteristics of traditional Chinese medicine(TCM)syndrome and identify potential biomarkers or intervention targets for DCM with heart failure toxin syndrome.Methods Fifteen male SD rats were divided into a blank control,doxorubicin,or DCM with heart failure toxin syndrome group using a random number table method,with five rats per group.The doxorubicin group received intraperitoneal injection of doxorubicin at a dose of 1.25 mg/kg,administered on the first and fourth days of each week,along with a standard diet.The DCM with heart failure toxin syndrome group,in addition to the doxorubicin treatment,was given 42%white liquor(10 mL/kg)via gavage every other day,along with a 45%high-fat feed and 10%fructose water.The blank control group received intraperitoneal injection of an equivalent volume of phosphate-buffered saline at the same time points as the doxorubicin group,along with a standard diet.The model was established for 10 weeks.At the fourth and tenth weeks of modeling,echocardiography was performed to measure left ventricular ejection fraction(LVEF),fractional shortening(FS),systolic left ventricular posterior wall thickness(LVPWs),diastolic left ventricular posterior wall thickness,systolic left ventricular internal diameter(LVIDs),and diastolic left ventricular internal diameter(LVIDd);macroscopic changes in fur color of the rats were assessed using the red-green-blue colorimetric method.After modeling,the open field test was conducted to evaluate the exercise tolerance of the rats,and the grip strength test was performed to assess changes in forelimb grip strength.Hematoxylin-eosin,Masson,and wheat germ agglutinin staining were used to evaluate pathological changes in cardiac tissue.Bulk RNA sequencing analysis was performed to identify differentially expressed genes(DEGs)in the hearts of rats between the blank control and the DCM with heart failure toxin syndrome groups.Using DCM,the Blue value of rat fur color,and forelimb grip strength as phenotypic traits,weighted gene co-expression network analysis(WGCNA)was performed to screen for characteristic module gene sets(MEs)associated with DCM with heart failure toxin syndrome.Overlapping analysis was performed on DEGs,immune-related gene sets,and MEs,and the intersecting genes were identified as potential biomarkers or intervention targets for DCM with heart failure toxin syndrome.The sensitivity and specificity of these targets were evaluated using receiver operating characteristic(ROC)curve analysis.Results Compared with the blank control group,at the tenth week of modeling,the LVEF,FS,and LVPWs of rats in the doxorubicin group and the DCM with heart failure toxin syndrome group decreased,whereas LVIDs and LVIDd increased,and the movement distance of the open field test and forelimb grip strength were reduced(P<0.05).At the 10th week of modeling,the Blue value of fur color in the DCM with heart failure toxin syndrome group was significantly lower than that of the blank control and doxorubicin groups(P<0.05).Compared with the blank control group,rats in the doxorubicin and DCM with heart failure toxin syndrome groups exhibited significant cardiac dilation and increased immune cell infiltration in cardiac tissue,accompanied by collagen deposition and cardiomyocyte hypertrophy.Bulk RNA sequencing identified 2,003 DEGs,including 1,082 downregulated genes and 921 upregulated genes.WGCNA results revealed that the MEturquoise module had the strongest positive correlation with DCM and the strongest negative correlation with the Blue value and forelimb grip strength.The overlapping analysis identified four intersecting genes:bone morphogenetic protein 6(Bmp6),serine-threonine-protein kinase 1(Pak1),proto-oncogene JunD(JunD),and S100 calcium-binding protein A3(S100A3).ROC curve analysis demonstrated that these four genes exhibited high sensitivity and specificity for DCM with heart failure toxin syndrome.Conclusion The rat model constructed by intraperitoneal injection of doxorubicin combined with a high-fat feed,fructose water,and white liquor gavage closely aligns with the characteristics of the DCM with heart failure toxin syndrome.Bmp6,JunD,Pak1,and S100A3 are potential biomarkers or therapeutic targets for DCM heart failure toxin syndrome.
5.Development and validation a predictive model for distinguishing malignant pleural effusion
Jinling JI ; Qiong WANG ; Ting SHI ; Yuzhang JIANG ; Chang LI
Chinese Journal of Clinical Laboratory Science 2025;43(9):702-709
Objective To development and validate a predictive model for distinguishing between malignant pleural effusion(MPE)and benign pleural effusion(BPE).Methods A total of 428 patients diagnosed with pleural effusion(PE)and hospitalized at the First Hospital of Huai'an Affiliated to Nanjing Medical University from July 2020 to May 2022 were selected.The patients were divided into BPE group(211 cases)and MPE group(217 cases)according to diagnostic criteria.The basic information and clinical data of these patients were collected.Boruta method was used for univariate screening,followed by multivariate Logistic regression to construct a basic nomogram model.Bootstrap method was used for internal validation to evaluate the performance of the nomogram,including dis-crimination,accuracy,and clinical applicability.Results The model included 8 key variables:dyspnea,chest pain,general symp-toms,X-ray/CT with malignant tumor features,serum carcinoembryonic antigen,serum neuron-specific enolase,pleural lactate dehy-drogenase,and pleural carcinoembryonic antigen.Internal validation showed that the area under the receiver operating characteristic curve(AUCROC)of the model was 0.933(95%confidence interval:0.912-0.954),with good accuracy(P>0.05).Decision curve a-nalysis(DCA)indicated that this predictive model for predicting MPE risk had a significant net benefit when the probability threshold exceeded 1%.Conclusion The constructed prediction model could effectively distinguish between MPE and BPE.
6.Pulmonary abscess caused by Slackia exigua:a case report and literature review
Yupei XIANG ; Bing FAN ; Jinling LI ; Xiaohong LI ; Jianhong WANG
Chinese Journal of Infection and Chemotherapy 2025;25(5):530-534
Objective To review the clinical characteristics of Slackia exigua infections for improving the diagnosis and treatment.Methods We reviewed the clinical characteristics of a case of pulmonary abscess caused by Slackia exigua.The infection cases caused by Slackia exigua were searched and retrieved from PubMed,CNKI,and Wanfang databases,for the articles published during the period from January 1,2011 to December 31,2023.Results Among the 15 cases of Slackia exigua infection,84.6%(11/13)had underlying diseases.Hypertension was the most common underlying disease,reported in 54.5%(6/11)of patients.Fever was present in 73.3%(11/15)of patients.Oral diseases and abscess caused by Slackia exigua were found in 63.6%(7/11)and 60.0%(9/15)of patients,respectively.Mixed infection was identified in 33.3%(5/15)of patients.The bacterium was susceptible to penicillin,ampicillin-sulbactam,ceftriaxone,levofloxacin and clindamycin,but mostly(2/3)resistant to metronidazole.Conclusions Slackia exigua infection often originates from oral aspiration and can cause abscess.Beta-lactam antibiotics are preferred for treatment of Slackia exigua infections.More data are required to confirm whether metronidazole is effective.
7.Analysis of Animal Models of Myasthenia Gravis Based on Its Clinical Characteristics in Chinese and Western Medicine
Yuhan CHEN ; Jinling CHEN ; Xin LI ; Yanhua OU ; Si WANG ; Jingyi CHEN ; Xingyi WANG ; Jiali YUAN ; Yuanyuan DUAN ; Zhongshan YANG ; Haitao NIU
Laboratory Animal and Comparative Medicine 2025;45(2):176-186
Myasthenia gravis(MG)is an autoimmune disease characterized primarily by skeletal muscle weakness and,in severe cases,respiratory involvement.Western medical treatment predominantly relies on immunosuppressants,but long-term administration often leads to notable side effects.In contrast,traditional Chinese medicine(TCM)offers the advantage of multi-target interventions.However,the pathogenesis of MG has not been fully elucidated,and the establishment of animal models that accurately reflect the clinical characteristics of both Chinese and Western medicine is essential for mechanism research and new drug development.This paper systematically reviews the etiology and pathogenesis,diagnostic criteria,and progress of animal model research for MG from both Chinese and Western medicine perspectives.In Western medicine,the pathogenesis of MG is closely related to genetic susceptibility,environmental factors,and autoantibody-mediated postsynaptic membrane damage.In TCM,MG is classified under the category of"flaccidity syndrome",attributed to congenital deficiencies and acquired malnourishment.Western diagnostic criteria involve a combination of clinical symptoms,fatigue testing,serum antibody assays,and electrophysiological evaluation.In contrast,TCM diagnosis emphasizes the integration of primary and secondary symptoms with tongue and pulse pattern differentiation.Currently available animal models mainly include experimental autoimmune myasthenia gravis(EAMG)and passive transfer myasthenia gravis(PTMG).The Toredo acetylcholine receptor(AChR)induced EAMG model aligns well with Western diagnostic criteria,but poorly matches secondary symptoms in TCM.The synthetic AChR peptide model is widely used,but shows low conformity with TCM syndromes.Models induced by muscle-specific tyrosine kinase(MuSK),low-density lipoprotein receptor-related protein 4(LRP4),and transgenic models demonstrate high innovation but exhibit low clinical conformity.Evaluation of these models requires integration of behavioral,electrophysiological,and immunological indicators.However,a systematic framework for modelling TCM syndromes is still lacking.Future research should integrate TCM-based etiological modelling methods with the Western pathological mechanisms to construct disease-syndrome combination models.Additionally,it is crucial to establish a TCM syndrome evaluation system based on"validation by prescription",as well as to improve the scientific rigor and practicality of animal models by the incorporation of emerging technologies.This review provides a theoretical foundation for optimizing MG animal model design,advancing the research on the combination of Chinese and Western medicine,and supporting efficacy assessment and mechanism exploration of Chinese herbal prescriptions.
8.Pulmonary abscess caused by Slackia exigua:a case report and literature review
Yupei XIANG ; Bing FAN ; Jinling LI ; Xiaohong LI ; Jianhong WANG
Chinese Journal of Infection and Chemotherapy 2025;25(5):530-534
Objective To review the clinical characteristics of Slackia exigua infections for improving the diagnosis and treatment.Methods We reviewed the clinical characteristics of a case of pulmonary abscess caused by Slackia exigua.The infection cases caused by Slackia exigua were searched and retrieved from PubMed,CNKI,and Wanfang databases,for the articles published during the period from January 1,2011 to December 31,2023.Results Among the 15 cases of Slackia exigua infection,84.6%(11/13)had underlying diseases.Hypertension was the most common underlying disease,reported in 54.5%(6/11)of patients.Fever was present in 73.3%(11/15)of patients.Oral diseases and abscess caused by Slackia exigua were found in 63.6%(7/11)and 60.0%(9/15)of patients,respectively.Mixed infection was identified in 33.3%(5/15)of patients.The bacterium was susceptible to penicillin,ampicillin-sulbactam,ceftriaxone,levofloxacin and clindamycin,but mostly(2/3)resistant to metronidazole.Conclusions Slackia exigua infection often originates from oral aspiration and can cause abscess.Beta-lactam antibiotics are preferred for treatment of Slackia exigua infections.More data are required to confirm whether metronidazole is effective.
9.Analysis of Animal Models of Myasthenia Gravis Based on Its Clinical Characteristics in Chinese and Western Medicine
Yuhan CHEN ; Jinling CHEN ; Xin LI ; Yanhua OU ; Si WANG ; Jingyi CHEN ; Xingyi WANG ; Jiali YUAN ; Yuanyuan DUAN ; Zhongshan YANG ; Haitao NIU
Laboratory Animal and Comparative Medicine 2025;45(2):176-186
Myasthenia gravis(MG)is an autoimmune disease characterized primarily by skeletal muscle weakness and,in severe cases,respiratory involvement.Western medical treatment predominantly relies on immunosuppressants,but long-term administration often leads to notable side effects.In contrast,traditional Chinese medicine(TCM)offers the advantage of multi-target interventions.However,the pathogenesis of MG has not been fully elucidated,and the establishment of animal models that accurately reflect the clinical characteristics of both Chinese and Western medicine is essential for mechanism research and new drug development.This paper systematically reviews the etiology and pathogenesis,diagnostic criteria,and progress of animal model research for MG from both Chinese and Western medicine perspectives.In Western medicine,the pathogenesis of MG is closely related to genetic susceptibility,environmental factors,and autoantibody-mediated postsynaptic membrane damage.In TCM,MG is classified under the category of"flaccidity syndrome",attributed to congenital deficiencies and acquired malnourishment.Western diagnostic criteria involve a combination of clinical symptoms,fatigue testing,serum antibody assays,and electrophysiological evaluation.In contrast,TCM diagnosis emphasizes the integration of primary and secondary symptoms with tongue and pulse pattern differentiation.Currently available animal models mainly include experimental autoimmune myasthenia gravis(EAMG)and passive transfer myasthenia gravis(PTMG).The Toredo acetylcholine receptor(AChR)induced EAMG model aligns well with Western diagnostic criteria,but poorly matches secondary symptoms in TCM.The synthetic AChR peptide model is widely used,but shows low conformity with TCM syndromes.Models induced by muscle-specific tyrosine kinase(MuSK),low-density lipoprotein receptor-related protein 4(LRP4),and transgenic models demonstrate high innovation but exhibit low clinical conformity.Evaluation of these models requires integration of behavioral,electrophysiological,and immunological indicators.However,a systematic framework for modelling TCM syndromes is still lacking.Future research should integrate TCM-based etiological modelling methods with the Western pathological mechanisms to construct disease-syndrome combination models.Additionally,it is crucial to establish a TCM syndrome evaluation system based on"validation by prescription",as well as to improve the scientific rigor and practicality of animal models by the incorporation of emerging technologies.This review provides a theoretical foundation for optimizing MG animal model design,advancing the research on the combination of Chinese and Western medicine,and supporting efficacy assessment and mechanism exploration of Chinese herbal prescriptions.
10.Effects of quality control circles combined with the strength concept in health education for pediatric leukemia patients
Chun GU ; Minhua WANG ; Jinling MA ; Rongrong LI
Chinese Journal of Modern Nursing 2025;31(2):253-257
Objective:To explore the effects of quality control circles (QCCs) combined with the strength concept in health education for pediatric leukemia patients.Methods:Totally 185 pediatric leukemia patients admitted to the Children's Hospital of Soochow University from January 2021 to August 2023 were selected as participants by convenience sampling. They were randomly divided into a control group ( n=92) and an observation group ( n=93) based on odd and even numbering. Patients in the control group received standard health education, while those in the observation group underwent QCC-based health education activities incorporating the strength concept. Dietary behavior improvement, treatment adherence, quality of life, and patient satisfaction with nursing care were compared between the two groups before and after the intervention. Results:After the intervention, dietary behavior scores in both groups decreased significantly, with the observation group showing significantly lower scores than the control group ( P<0.05). Treatment adherence and quality of life scores improved significantly in both groups, with the observation group scoring higher than the control group across all dimensions ( P<0.05). Moreover, the observation group demonstrated higher patient satisfaction with nursing care compared to the control group ( P<0.05) . Conclusions:The application of QCC activities combined with health education based on the strength concept effectively improves dietary behavior, treatment adherence, quality of life, and satisfaction with nursing care among pediatric leukemia patients, which is worth promoting in clinical practice.


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