1.Research Progress on Qingwen Baidu Decoction and Its Active Ingredients in Prevention of Lung Injury
Xiaojie LIN ; Xianan WEN ; Qiaolin ZENG ; Li WANG ; Yuanru ZHENG ; Jie ZHAO
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(9):315-322
Acute lung injury (ALI) is a common and highly lethal clinical syndrome characterized by acute progressive respiratory failure. Currently, the treatment of ALI primarily involves respiratory support therapy and symptomatic pharmacotherapy, yet there is still a lack of specific and effective pharmacological treatments. Qingwen Baidu decoction is a traditional Chinese medicine formula that has the effects of clearing heat, removing toxin, cooling blood, and purging fire. Its pharmacological effects include anti-inflammatory, antipyretic, antibacterial, antiviral, sedative, and so on. The flavonoids, phenols, terpenes, and other components contained in this formula have strong pharmacological activity, which can regulate the inflammatory response caused and oxidative stress in ALI and maintain the integrity of alveolar-capillary barrier (ACB) by anti-apoptosis, anti-pathogen infection, and anti-pulmonary fibrosis, thereby improving the pathological changes of lung tissue. Among them, flavonoids have been reported more, and their mechanism of action is complex and diverse. For example, quercetin, luteolin, and baicalin act on multiple important targets, such as signal transducer and activator of transcription 3 (STAT3), mitogen-activated protein kinase 3 (MAPK3), etc. and participate in the regulation of Toll-like receptor 4 (TLR4)/myeloid differentiation primary response 88 (MyD88)/nuclear factor kappa B (NF-κB), nuclear factor erythroid 2-related factor 2(Nrf2)/Kelch-like ECH-associated protein 1 (Keap1), and silent information regulator 1 (SIRT1)/forkhead box protein O1 (FoxO1) signaling pathways, thereby intervening in pathological events such as inflammation, oxidative stress, cell apoptosis, and fibrosis. This paper aims to review the research progress on Qingwen Baidu decoction and its active ingredients in the prevention and treatment of lung injury in the expectation of providing reference for its subsequent pharmacological mechanism research and theoretical support for its clinical application and drug development in the treatment of ALI.
2.Progress of the application of deep learning in degenerative cervical myelopathy
Qian-Bo SONG ; Qian DU ; Yan ZENG ; Yuan-Ming LU ; Wen-Xing LIAO ; Dong ZHAO ; Guang-Ru CAO
Medical Journal of Chinese People's Liberation Army 2025;50(10):1256-1262
Degenerative cervical myelopathy(DCM)is a group of diseases caused by cervical spine degeneration that compresses the spinal cord.It is a major cause of spinal cord dysfunction in adults,and its incidence is increasing globally.In the late stage,DCM could lead to paralysis due to spinal cord injury,which makes rapid,effective,and accurate medical diagnosis clinically significant.Deep learning(DL)technology can assist physicians in the rapid and accurate diagnosis of DCM by analyzing and processing a large amount of imaging data to extract features of the affected regions.In recent years,DL algorithm models have been leveraged for DCM-related research,which has become a focal point of intelligent medical development.In this review,domestic and international literature is surveyed,and the research progress and application of DL technology in the auxiliary diagnosis and prognosis evaluation of DCM are systematically summarized,aiming to provide a reference for intelligent diagnosis in clinical practice.
3.Traditional Chinese medicine dry powder inhalers: research status and development ideas and methods.
Yu-Wen MA ; Yi-Chen ZENG ; Hao-Ran WANG ; Guang-Fu LIU ; Jun JIANG ; Yu-Song ZENG ; Bai-Xiu ZHAO ; Jin FANG
China Journal of Chinese Materia Medica 2025;50(3):620-631
As an innovative dosage form, traditional Chinese medicine(TCM) dry powder inhalers have emerged as a focal point in the research and development of new preparations due to its high efficiency, safety, and bioavailability. This paper systematically reviewed the relevant literature and patents associated with TCM dry powder inhalers to analyze the origins and the current research and development status. Furthermore, this paper probed into the research and development ideas of TCM dry powder inhalers regarding clinical positioning, prescription screening, and druggability. Additionally, the paper thoroughly analyzed the technical barriers in druggability studies and elaborated on corresponding research techniques and coping measures. Furthermore, it emphasized the need for improved regulations and policies governing TCM dry powder inhalers, advocated for strengthened oversight, and called for the establishment of a scientific quality evaluation system. Measures such as promoting production-education-research collaboration, enhancing personnel training, and fostering international exchanges were proposed to provide a scientific and systematic reference for the future research, development, and application of TCM dry powder inhalers, thereby facilitating the rapid modernization of TCM.
Humans
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Dry Powder Inhalers/trends*
;
Drugs, Chinese Herbal/chemistry*
;
Medicine, Chinese Traditional/instrumentation*
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Administration, Inhalation
4.Mediating effect of sleep duration between depression symptoms and myopia in middle school students.
Wei DU ; Xu-Xiang YANG ; Ru-Shuang ZENG ; Chun-Yao ZHAO ; Zhi-Peng XIANG ; Yuan-Chun LI ; Jie-Song WANG ; Xiao-Hong SU ; Xiao LU ; Yu LI ; Jing WEN ; Dang HAN ; Qun DU ; Jia HE
Chinese Journal of Contemporary Pediatrics 2025;27(3):359-365
OBJECTIVES:
To explore the mediating role of sleep duration in the relationship between depression symptoms and myopia among middle school students.
METHODS:
This study was a cross-sectional research conducted using a stratified cluster random sampling method. A total of 1 728 middle school students were selected from two junior high schools and two senior high schools in certain urban areas and farms of the Xinjiang Production and Construction Corps. Questionnaire surveys and vision tests were conducted among the students. Spearman analysis was used to analyze the correlation between depression symptoms, sleep duration, and myopia. The Bootstrap method was employed to investigate the mediating effect of sleep duration between depression symptoms and myopia.
RESULTS:
The prevalence of myopia in the overall population was 74.02% (1 279/1 728), with an average sleep duration of (7.6±1.0) hours. The rate of insufficient sleep was 83.62% (1 445/1 728), and the proportion of students exhibiting depression symptoms was 25.29% (437/1 728). Correlation analysis showed significant negative correlations between visual acuity in both eyes and sleep duration with depressive emotions as measured by the Center for Epidemiologic Studies Depression Scale (with correlation coefficients of -0.064, -0.084, and -0.199 respectively; P<0.01), as well as with somatic symptoms and activities (with correlation coefficients of -0.104, -0.124, and -0.233 respectively; P<0.01) and interpersonal relationships (with correlation coefficients of -0.052, -0.059, and -0.071 respectively; P<0.05). The correlation coefficients for left and right eye visual acuity and sleep duration were 0.206 and 0.211 respectively (P<0.001). Sleep duration exhibited a mediating effect between depression symptoms and myopia (indirect effect=0.056, 95%CI: 0.029-0.088), with the mediating effect value for females (indirect effect=0.066, 95%CI: 0.024-0.119) being higher than that for males (indirect effect=0.042, 95%CI: 0.011-0.081).
CONCLUSIONS
Sleep duration serves as a partial mediator between depression symptoms and myopia in middle school students.
Humans
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Myopia/etiology*
;
Male
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Female
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Depression/physiopathology*
;
Cross-Sectional Studies
;
Sleep
;
Adolescent
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Students
;
Child
;
Time Factors
;
Sleep Duration
5.Effects of Electroacupuncture at "Fengchi" (GB 20), "Waiguan" (TE 5), and "Yanglingquan" (GB 34) on Nociceptive Sensitization and PKC/TRPV1 Pathway in the Trigeminal Ganglion of Chronic Migraine Model Rats
Yixiang ZENG ; Runze TU ; Shucong ZHAO ; Yang YANG ; Haojia WEN ; Zhuozhong HE ; Shengli ZHOU ; Lei TAN ; Ke HE ; Lei FU
Journal of Traditional Chinese Medicine 2025;66(3):283-289
ObjectiveTo explore the possible mechanisms of electroacupuncture at Fengchi (GB 20), Waiguan (TE 5), and Yanglingquan (GB 34) in treating chronic migraine from the perspective of nociceptive sensitization. MethodsForty SPF-grade SD rats were randomly divided into blank group, model group, electroacupuncture group, electroacupuncture + agonist group, and inhibitor group, with 8 rats in each group. Except for the blank group, rats were injected intraperitoneally with nitroglycerin to establish a chronic migraine rat model. After successful modeling, the electroacupuncture group received electroacupuncture at bilateral "Fengchi" (GB 20), "Waiguan" (TE 5), and "Yanglingquan" (GB 34) for 30 minutes each session. The electroacupuncture + agonist group received the same electroacupuncture treatment and additional injection of protein kinase C (PKC) agonist Phorbol 12-myristate 13-acetate (1.0 ng/μl, 25 μl) via the infraorbital foramen. The inhibitor group received PKC inhibitor Chelerythrine Chloride (1.0 ng/μl, 10 μl) via the infraorbital foramen. The blank group, model group, and inhibitor group underwent restraint for 30 minutes without other interventions. All groups were continuously intervened for 5 days. After the intervention, the nociceptive thresholds (mechanical and thermal pain) of the periorbital area and hind paw were measured. The expression levels of transient receptor potential vanillic acid subtype 1 (TRPV1), phosphorylated TRPV1 (p-TRPV1), PKC proteins, Trpv1, Pkc mRNA, and the average fluorescence intensity of transient receptor potential vanillic acid subtype 1 (TRPV1) and PKC in the trigeminal ganglion were detected using Western Blot, real-time fluorescence quantitative PCR, and immunofluorescence methods. ResultsCompared with the blank group, the mechanical and thermal pain thresholds of the periorbital area and hind paw were reduced in the model group, and the protein levels of TRPV1, PKC, p-TRPV1, as well as the mRNA expression of Trpv1 and Pkc, and the average fluorescence intensity of TRPV1 and PKC in the trigeminal ganglion significantly increased (P<0.05 or P<0.01). Compared with the model group, the electroacupuncture group exhibited increased mechanical and thermal pain thresholds in the periorbital and hind paw areas, and decreased protein levels of TRPV1, PKC, p-TRPV1, mRNA expression of Trpv1 and Pkc, and average fluorescence intensity of TRPV1. In the electroacupuncture + agonist group, the average fluorescence intensity of TRPV1 in the trigeminal ganglion decreased. The inhibitor group exhibited increased mechanical pain thresholds in the periorbital area and thermal pain thresholds in the hind paw, along with decreased protein levels of TRPV1, PKC, p-TRPV1, and the average fluorescence intensity of TRPV1 and PKC (P<0.05 or P<0.01). Compared with the electroacupuncture group, the electroacupuncture + agonist group showed an increase in the protein levels of TRPV1, PKC, p-TRPV1, and the mRNA expression of Trpv1 (P<0.05 or P<0.01). ConclusionElectroacupuncture at the "Fengchi" (GB 20), "Waiguan" (TE 5), and "Yanglingquan" (GB 34) acupoints can increase the mechanical and thermal pain thresholds in chronic migraine rats and alleviate nociceptive sensitization. The mechanism may be related to the inhibition of PKC/TRPV1 pathway.
6.Risk factors for bronchopulmonary dysplasia in twin preterm infants:a multicenter study
Yu-Wei FAN ; Yi-Jia ZHANG ; He-Mei WEN ; Hong YAN ; Wei SHEN ; Yue-Qin DING ; Yun-Feng LONG ; Zhi-Gang ZHANG ; Gui-Fang LI ; Hong JIANG ; Hong-Ping RAO ; Jian-Wu QIU ; Xian WEI ; Ya-Yu ZHANG ; Ji-Bin ZENG ; Chang-Liang ZHAO ; Wei-Peng XU ; Fan WANG ; Li YUAN ; Xiu-Fang YANG ; Wei LI ; Ni-Yang LIN ; Qian CHEN ; Chang-Shun XIA ; Xin-Qi ZHONG ; Qi-Liang CUI
Chinese Journal of Contemporary Pediatrics 2024;26(6):611-618
Objective To investigate the risk factors for bronchopulmonary dysplasia(BPD)in twin preterm infants with a gestational age of<34 weeks,and to provide a basis for early identification of BPD in twin preterm infants in clinical practice.Methods A retrospective analysis was performed for the twin preterm infants with a gestational age of<34 weeks who were admitted to 22 hospitals nationwide from January 2018 to December 2020.According to their conditions,they were divided into group A(both twins had BPD),group B(only one twin had BPD),and group C(neither twin had BPD).The risk factors for BPD in twin preterm infants were analyzed.Further analysis was conducted on group B to investigate the postnatal risk factors for BPD within twins.Results A total of 904 pairs of twins with a gestational age of<34 weeks were included in this study.The multivariate logistic regression analysis showed that compared with group C,birth weight discordance of>25%between the twins was an independent risk factor for BPD in one of the twins(OR=3.370,95%CI:1.500-7.568,P<0.05),and high gestational age at birth was a protective factor against BPD(P<0.05).The conditional logistic regression analysis of group B showed that small-for-gestational-age(SGA)birth was an independent risk factor for BPD in individual twins(OR=5.017,95%CI:1.040-24.190,P<0.05).Conclusions The development of BPD in twin preterm infants is associated with gestational age,birth weight discordance between the twins,and SGA birth.
7.Expression of UBE2C in liver cancer tissues and its effect on proliferation and invasion of hepatocellular carcinoma cells HepG2
Ping ZHAO ; Dan PANG ; Xi-Zeng ZHU ; Hai-Xia SHAN ; Hui-Wen JIA
Chinese Journal of Current Advances in General Surgery 2024;27(7):511-516
Objective:To investigate the expression level of UBE2C in liver cancer tissues and its effect on the proliferation and invasion of hepatocellular carcinoma cells HepG2 after UBE2C silencing.Methods:The data set of liver cancer was downloaded from the TCGA database.Ac-cording to the median expression level of UBE2C mRNA in liver cancer tissues,all liver cancer pa-tients were divided into UBE2C higher(n=169)and lower expression group(n=205),respectively.The expression level of UBE2C mRNA in liver cancer tissues and its relationship with the patients prognosis was analyzed.COX regression was used to analyze the influencing factors of the liver cancer patients prognosis.The human hepatocellular carcinoma cell lines(HepG2,Huh7 and SMMC-7721)and human nromal hepatic epithelial cell line(THLE-3)were selected,and the ex-pression level of UBE2C in the four cell lines were detected by Western blot and real-time fluores-cence quantitative PCR,respectively.The HepG2 cell line was protein and mRNA expression leves divided into control group,NC group and si-UBE2C group according to UBE2C silencing.The ef-fects of UBE2C silencing on proliferation and invasion of HepG2 cell line were analyzed.Results:The expression level of UBE2C mRNA in liver cancer tissues and adjacent normal liver tissues were 4.342(3.239,5.635)and 0.905(0.587,1.230),respectively.Compared with adjacent normal liver tissues,UBE2C mRNA levels in liver cancer tissues were significantly higher(P<0.001).The UBE2C mRNA expression levels in liver cancer tissues and paired adjacent normal liver tissues were 4.266(3.342,5.054)and 0905(0.587,1.230),respectively.Compared with paired adjacent normal liver tissues,UBE2C mRNA expression levels in liver cancer tissues were significantly higher(P<0.001).The median survival time of UBE2C mRNA higher and lower expression groups was 48.85 months and 69.38 months.Compared with the lower expression group,the median survival time of UBE2C mRNA higher expression group was significantly shortened(P=0.045).T staging(T3/T4)and UBE2C expression(higher expression)were independent risk factors for poor prognosis in patients with liver cancer(P<0.05).Compared with human liver epithelial cell line THLE-3,UBE2C protein and mRNA were significantly higher expressed in human hepatocellular carcinoma cell line HepG2,Huh7 and SMMC-7721(P<0.05).The expression level of UBE2C protein and mRNA expression was the most significant in human hepatocellular carcinoma cell line HepG2 relative to cell line Huh7 and SMMC-7721.The CCK-8 results show that the cell proliferation rate in si-UBE2C group were significantly decreased protein and mRNA expression levels compared to control group and NC group at 72 h and 96 h,and the differences were significant(P<0.05).The number of invasive cells in control group,NC group and si-UBE2C group were(23.12±3.45),(24.33±2.83)and(10.21±1.14),respectively.Compared with control group and NC group,the number of invasive cells in si-UBE2C group was significantly decreased(P<0.05).Conclusion:UBE2C was higher expressed in liver cancer,and can be used as a biomarker for poor prognosis of patients with liver cancer.After silencing of UBE2C gene can significantly inhibit proliferation and invasion of HepG2.
8.Study of lncRNA-miRNA-mRNA ceRNA regulatory network mediated by serum exosomes in coronary heart disease and prediction and experimental validation of potential target herbal medicines
Lu MA ; Lei YANG ; Huang DING ; Wan-Yu LI ; Wei TAN ; Yan-Ling LI ; Yan-Yan ZHANG ; Xiao-Dan LIU ; Zhao-Wen ZENG ; Chang-Qing DENG ; Wei ZHANG
Chinese Pharmacological Bulletin 2024;40(6):1153-1164
Aim To analyze serum exosome sequencing data from patients with coronary heart disease(CHD)and normal subjects by using bioinformatics-related methods to construct a competitive endogenous ln-cRNA-miRNA-mRNA(ceRNA)regulatory network,to mine the predicted potential Chinese medicines,and to perform preliminary validation of the biological processes and core Chinese medicines involved in the ceRNA network.Methods We used exoRbase data-base to obtain the expression matrix of differential genes,combined with the raw letter method to con-struct the ceRNA network,and performed GO analysis and KEGG analysis on the differential mRNAs in the network,and used COREMINE database to predict the biological processes and core target genes involved in the ceRNA network,and to screen the herbal medi-cines with potential therapeutic effects;AVECs oxida-tive damage cell model was constructed in vitro,and the cytoskeleton,tube-forming function,cell prolifera-tion,LDH leakage rate,ROS level and p-AKT,AKT,p-PI3K and AKT protein expression were examined to verify the action pathways and targets of the core Chi-nese medicine Salvia miltiorrhiza for the treatment of coronary heart disease.Results Compared with nor-mal subjects,395 mRNAs,80 miRNAs,60 lncRNA differential genes,and 80 miRNAs were predicted in serum exosomes of coronary heart disease,and the constructed ceRNA sub-network,mainly consisted of 21 lncRNAs,80 miRNAs,and 82 mRNAs;AKT1,VEGFA,IL1B and other genes in the network.The abnormally expressed mRNAs were involved in biologi-cal processes such as oxidative stress and signaling pathways such as PI3 K/Akt,and Dan Shen,Chuanx-iong and Panax notoginseng were most closely related to exosome-mediated biological processes and core genes in coronary heart disease.The active ingredients of tanshinone ⅡA,the core Chinese medicine,could pro-mote vascular endothelial cell proliferation,tube for-mation,skeleton formation and repair,reduce LDH leakage rate and ROS level,and promote the expres-sion of p-AKT and p-PI3K protein.Conclusion There is a complex ceRNA regulatory network trans-duction in coronary artery disease serum exosomes,and traditional Chinese medicine can be used to treat CHD through multi-target intervention,and Dan Shen,Chuanxiong and Panax notoginseng are expected to be candidate sources of traditional Chinese medicine,a-mong which the active ingredient of Dan Shen,tanshi-none ⅡA,activates PI3 K/Akt signaling pathway to play a protective role against oxidative stress-injured cells,and treats CHD.
9.Analysis of clinical characteristics and influencing factors of vascular involvement in Beh?et′s disease
Dongmei PAN ; Cheng ZHAO ; Zhanrui CHEN ; Fang QIN ; Jing WEN ; Wanling WEI ; Wen ZENG ; Xiaoling LIAO ; Fei DONG ; Ling LEI
Chinese Journal of Rheumatology 2024;28(8):566-570
Objective:To analyze the clinical characteristics and influencing factors of vascular involvement in Beh?et′s Disease (BD) to improve and provideunderstanding of insights for clinicians to better understand this condition.Methods:Clinical data from 220 BD patients admitted to the First Affiliated Hospital of Guangxi Medical University from January 2012 to May 2022 were collected. Clinical manifestations and laboratory findings were compared between BD patients with and without vascular involvement, as well as between those with improved conditions and those with progressive conditions. Binary logistic regression was used to analyze the influencing factors.Results:①The average age of the 220 BD patients was 36.5±15.3 years. Among them, 23 patients (10.5%) had vascular involvement, including 20 males (87.0%).②Compared to BD patients without vascular involvement, those with vascular involvement had significantly higher rates of smoking [6.1%(12/197) vs.34.8%(8/23), χ2=17.19, P<0.001], cardiac involvement [1.5%(3/197) vs. 13.0%(9/23), χ2=6.42, P=0.011], and elevated C-reactive protein(CRP) levels (78.3% vs. 56.3%, χ2=4.08, P=0.043).③ Among BD patients with vascular involvement, 11 cases (47.8%) had venous lesions, and 20 cases (87.0%) had arterial lesions, with 8 cases (34.8%) having both venous and arterial involvement. The most common type of vascular involvement was arterial dilatation (11 cases), mainly aneurysms (10 cases), and deep venous thrombosis of the lower extremities (7 cases).④The 23 BD patients with vascular involvement were followed up for an average of 18.3 months. Among them, 16 patients (69.6%) showed stable improvement, while 7 patients (30.4%) experienced disease progression, including 4 deaths (1 male and 3 females). A total of 91.3% (21/23) of the patients received glucocorticoid therapy. Immunosuppressive therapy was administered to 82.6% (19/23) of the patients, with 65.2% (10/23) receiving with cyclophosphamide and 43.5% receiving with thalidomide. Additionally, 13% (3/23) of the patients were treated with cyclosporine and methotrexate, respectively, and 8.7% (2/23) were treated with received mycophenolate mofetil. Anticoagulant therapy was given to 21.7% (5/23) of the patients, using either warfarin or low molecular weight heparin. Biologic therapy was administered to 17.4% (4/23) of the patients, and surgical intervention was performed in 43.5% (10/23) of the patients. ⑤Binary logistic regression analysis identified male gender [ OR(95% CI)=5.70(1.60, 20.90), P=0.009] as an indepe-ndent risk factor for vascular involvement in BD. Conclusion:The incidence of vascular involvement in BD is 10.5%, with a higher prevalence in males. Arterial involvement is more common than venous involvement, with arterial aneurysms being the most common manifestation. Clinicians should pay attention to CRP and total cholesterol levels in BD patients.
10.Clinical features of hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma: a multi-center real-world retrospective study
Yunze XU ; Wen KONG ; Ming CAO ; Guangxi SUN ; Jinge ZHAO ; Songyang LIU ; Zhiling ZHANG ; Liru HE ; Xiaoqun YANG ; Haizhou ZHANG ; Lieyu XU ; Yanfei YU ; Hang WANG ; Honggang QI ; Tianyuan XU ; Bo YANG ; Yichu YUAN ; Dongning CHEN ; Dengqiang LIN ; Fangjian ZHOU ; Qiang WEI ; Wei XUE ; Xin MA ; Pei DONG ; Hao ZENG ; Jin ZHANG
Chinese Journal of Urology 2024;45(3):161-167
Objective:To investigate the clinical features and therapeutic efficacy of patients with hereditary leiomyomatosis and renal cell carcinoma(RCC) syndrome-associated RCC (HLRCC-RCC) in China.Methods:The clinical data of 119 HLRCC-RCC patients with fumarate hydratase (FH) germline mutation confirmed by genetic diagnosis from 15 medical centers nationwide from January 2008 to December 2021 were retrospectively analyzed. Among them, 73 were male and 46 were female. The median age was 38(13, 74) years. The median tumor diameter was 6.5 (1.0, 20.5) cm. There were 38 cases (31.9%) in stage Ⅰ-Ⅱand 81 cases (68.1%) in stage Ⅲ-Ⅳ. In this group, only 11 of 119 HLRCC-RCC patients presented with skin smooth muscle tumors, and 44 of 46 female HLRCC-RCC patients had a history of uterine fibroids. The pathological characteristics, treatment methods, prognosis and survival of the patients were summarized.Results:A total of 86 patients underwent surgical treatment, including 70 cases of radical nephrectomy, 5 cases of partial nephrectomy, and 11 cases of reductive nephrectomy. The other 33 patients with newly diagnosed metastasis underwent renal puncture biopsy. The results of genetic testing showed that 94 patients had FH gene point mutation, 18 had FH gene insertion/deletion mutation, 4 had FH gene splicing mutation, 2 had FH gene large fragment deletion and 1 had FH gene copy number mutation. Immunohistochemical staining showed strong 2-succinocysteine (2-SC) positive and FH negative in 113 patients. A total of 102 patients received systematic treatment, including 44 newly diagnosed patients with metastasis and 58 patients with postoperative metastasis. Among them, 33 patients were treated with tyrosine kinase inhibitor (TKI) combined with immune checkpoint inhibitor (ICI), 8 patients were treated with bevacizumab combined with erlotinib, and 61 patients were treated with TKI monotherapy. Survival analysis showed that the median progression-free survival (PFS) of TKI combined with ICI was 18 (5, 38) months, and the median overall survival (OS) was not reached. The median PFS and OS were 12 (5, 14) months and 30 (10, 32) months in the bevacizumab combined with erlotinib treatment group, respectively. The median PFS and OS were 10 (3, 64) months and 44 (10, 74) months in the TKI monotherapy group, respectively. PFS ( P=0.009) and OS ( P=0.006) in TKI combined with ICI group were better than those in bevacizumab combined with erlotinib group. The median PFS ( P=0.003) and median OS ( P=0.028) in TKI combined with ICI group were better than those in TKI monotherapy group. Conclusions:HLRCC-RCC is rare but has a high degree of malignancy, poor prognosis and familial genetic characteristics. Immunohistochemical staining with strong positive 2-SC and negative FH can provide an important basis for clinical diagnosis. Genetic detection of FH gene germ line mutation can confirm the diagnosis. The preliminary study results confirmed that TKI combined with ICI had a good clinical effect, but it needs to be confirmed by the results of a large sample multi-center randomized controlled clinical study.

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