1.Analysis of pediatric pre-prescription review orders based on PCNE classification system
Anle SHEN ; Peiqi WANG ; Tao XU ; Jia LUO ; Xuexian WANG ; Shunguo ZHANG ; Zhiling LI
China Pharmacy 2025;36(3):351-355
OBJECTIVE To provide reference for improving the pre-prescription review system and reducing the occurrence of medication error by analyzing the drug-related problems (DRPs) in the pre-prescription review orders of pediatric outpatient clinics using the Pharmaceutical Care Network Europe (PCNE) classification system. METHODS The data of pre-prescription review orders were retrospectively collected from outpatient department of Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine from July 2022 to June 2023; DRPs in the pre-prescription review orders were classified and summarized by using the PCNE classification system (version 9.1), and then analyzed in terms of types and causes of issues, and the acceptance of interventions. RESULTS A total of 66 017 DRPs orders were included, involving 41 165 patients. The proportion of DRPs orders in children aged ≤5 years old was the highest (58.25%), followed by children aged 6-12 years old (33.52%); the department with the highest proportion of DRPs was internal medicine of pediatrics department (71.41%); the department with the highest incidence of DRPs was thoracic surgery department (9.73%); top three drug categories of DRPs orders were systemic anti- infective drugs (25.26%), Chinese patent medicines (24.74%) and respiratory drugs (22.38%). Referring to PCNE classification system, the types of DRPs mainly focused on treatment safety (64.86%); the reasons of DRPs orders mainly focused on dose selection (82.09%), of which 41.26% were due to excessive drug dosage; 92.13% of interventions could be accepted and fully executed by doctors. CONCLUSIONS DRPs orders identified by the pre-prescription review system can be effectively analyzed by using PCNE classification system. Pharmacists should focus on medication use in children aged ≤5 years old, update and develop personalized prescription review rules timely, and meet the rational needs of clinical medication for children.
2.Integrated plasma and synovial membrane lipidomic profiling revealing the therapeutic effects of moxibustion in collagen-induced arthritis rat models
Jiamin WEN ; Rui ZHANG ; Danwen WANG ; Zhiling SUN
Digital Chinese Medicine 2025;8(2):254-266
Objective:
To reveal the therapeutic effects of moxibustion in collagen-induced arthritis (CIA) rat models using the combined analysis of plasma and synovial membrane lipidomic profiling and to enhance the understanding of how moxibustion affects lipid metabolism in rheumatoid arthritis (RA).
Methods:
A total of 32 male Sprague-Dawley (SD) rats were randomly assigned to four groups: control, moxibustion control (MC), model, and moxibustion model (MM) groups, with 8 rats in each group. CIA was induced in SD rats by two immunizations. The paw volume was measured before the induction of CIA. Following induction, after assessing paw volume and arthritis index (AI) scores, the MC and MM groups received treatment at bilateral Shenshu (BL23) and Zusanli (ST36) acupoints for 10 min per acupoint. The intervention included three treatment courses, each spanning 6 d and followed by a 1-d interval. Paw volume and AI scores were assessed after each treatment course. After the completion of the three treatment courses, serum, plasma, synovial tissue, and ankle joint samples were collected. Enzyme-linked immunosorbent assay (ELISA) was employed to quantify the levels of interleukin (IL)-6 and tumor necrosis factor (TNF)-α in serum. Hematoxylin and eosin (HE) staining was performed for histopathological examination of the ankle joint tissues. Meanwhile, ultra-high-performance liquid chromatography coupled with Q-Exactive Orbitrap mass spectrometry (UHPLC-Q-Exactive Orbitrap MS) was utilized to analyze the plasma and synovial tissue samples. In addition, multivariate statistical analysis was performed to identify differential lipid metabolites, and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis was applied to explore metabolic pathways modulated by moxibustion therapy.
Results:
No significant difference in hind paw volume and AI scores was observed among the groups (P > 0.05). After CIA induction, model group showed increased hind paw volume and AI scores compared with control group (P < 0.05), which were significantly reduced after moxibustion treatment in MM group compared with model group (P < 0.05). The levels of IL-6 and TNF-α were significantly higher in model and MM groups compared with control group (P < 0.05), but were lower in MM group than those in model group (P < 0.05). Histopathological analysis showed improved cartilage and reduced inflammation in MM group. A total of 33 differential lipid metabolites in the plasma and 24 in the synovial membranes of CIA rat models were identified when compared with control group. Among these lipid metabolites, 31 in the plasma and all 24 in the synovial membranes were regulated by moxibustion treatment. Pathological analysis revealed upregulation of diacylglycerol (DG) and fatty acid (FA) levels, alongside downregulation of lysophosphatidylcholine (LPC), phosphatidylcholine (PC), and phosphatidylethanolamine (PE). Under physiological conditions, the treatment specifically reduced LPC and PC levels. Pathway enrichment analysis revealed that moxibustion predominantly affected α-linolenic acid, glycerophospholipid, and sphingolipid metabolism under pathological conditions. Under physiological conditions, the regulation was centered around α-linolenic acid and glycerophospholipid metabolism.
Conclusion
The RA rat models exhibited significant lipid metabolic disturbances. Moxibustion alleviated paw swelling, reduced AI scores, modulated inflammatory cytokine levels, and partially corrected the altered levels of multiple lipid metabolites. The potential metabolic pathways implicated in the regulation of lipid metabolism under both physiological and pathological conditions include α-linolenic acid, glycerophospholipid, and sphingolipid metabolism.
3.Integrated imaging and clinical features of glottic squamous cell carcinoma of the larynx: pathological association and prognosis assessment.
Yuqiao ZHANG ; Wulin WEN ; Fengxia YANG ; Dongke MA ; Xueliang SHEN ; Ningyu FENG ; Xixi LI ; Zhiling ZENG ; Zhipeng MI ; Xiyuan YAN ; Ruixia MA
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2025;39(8):709-716
Objective:To explore the clinical, imaging, and pathological features of glottic squamous cell carcinoma of the larynx and their relationship with prognosis. Methods:A retrospective analysis was conducted on the clinical, imaging, and pathological data of 130 patients with glottic squamous cell carcinoma of the larynx who were treated at the First People's Hospital of Yinchuan and the General Hospital of Ningxia Medical University from January 2018 to March 2023. Imaging examinations (CT and MRI) were used to evaluate the lesion boundary clarity, density, enhancement nature, and enhancement degree. Postoperative pathological examination was used to determine the pathological nature, immunohistochemistry, etc. Statistical methods such as χ² test, Spearman correlation analysis, multivariate logistic regression analysis, and Kaplan-Meier method were used to analyze the data. Results:Among the 130 patients, 127 were male and 3 were female, with an average age of (61.92±9.595) years. There was a correlation between clinical, imaging, and pathological features. Multivariate analysis showed that heterogeneous MRI density (OR=12.414;P=0.019) and squamous cell carcinoma as a subtype were correlated. The initial symptom of non-hoarseness (HR=6.045;P=0.010) and unclear MRI boundary (HR=12.559; P=0.029) were independent risk factors for poor prognosis in patients with glottic squamous cell carcinoma of the larynx. Conclusion:There is a correlation between the clinical, imaging, and pathological features of patients with glottic squamous cell carcinoma of the larynx, and they can affect prognosis. The initial symptom of non-hoarseness and unclear MRI boundary of the tumor are independent risk factors for poor prognosis.
Humans
;
Laryngeal Neoplasms/diagnosis*
;
Prognosis
;
Male
;
Female
;
Retrospective Studies
;
Middle Aged
;
Carcinoma, Squamous Cell/diagnosis*
;
Magnetic Resonance Imaging
;
Glottis/pathology*
;
Tomography, X-Ray Computed
;
Aged
4.Predictive value of oxygenation index at intensive care unit admission for 30-day mortality in patients with sepsis.
Chunhua BI ; Manchen ZHU ; Chen NI ; Zongfeng ZHANG ; Zhiling QI ; Huanhuan CHENG ; Zongqiang LI ; Cuiping HAO
Chinese Critical Care Medicine 2025;37(2):111-117
OBJECTIVE:
To investigate the predictive value of oxygenation index (PaO2/FiO2) at intensive care unit (ICU) admission on 30-day mortality in patients with sepsis.
METHODS:
A retrospective study was conducted. Patients with sepsis who were hospitalized in the ICU of the Affiliated Hospital of Jining Medical University from April 2015 to October 2023 were enrolled. The demographic information, comorbidities, sites of infection, vital signs and laboratory test indicators at the time of admission to the ICU, disease severity scores within 24 hours of admission to the ICU, treatment process and prognostic indicators were collected. According to the PaO2/FiO2 at ICU admission, patients were divided into Q1 group (PaO2/FiO2 of 4.1-16.4 cmHg, 1 cmHg ≈ 1.33 kPa), Q2 group (PaO2/FiO2 of 16.5-22.6 cmHg), Q3 group (PaO2/FiO2 of 22.7-32.9 cmHg), and Q4 group (PaO2/FiO2 of 33.0-94.8 cmHg). Differences in the indicators across the four groups were compared. Multifactorial Cox regression analysis was used to assess the relationship between PaO2/FiO2 and 30-day mortality of patients with sepsis. The predictive value of PaO2/FiO2, sequential organ failure assessment (SOFA) and acute physiology and chronic health evaluation II (APACHE II) on 30-day prognosis of patients with sepsis was analyzed by receiver operator characteristic curve (ROC curve).
RESULTS:
A total of 1 711 patients with sepsis were enrolled, including 428 patients in Q1 group, 424 patients in Q2 group, 425 patients in Q3 group, and 434 patients in Q4 group. 622 patients died at 30-day, the overall 30-day mortality was 36.35%. There were statistically significant differences in age, body mass index (BMI), history of smoking, history of alcohol consumption, admission heart rate, respiratory rate, APACHE II score, SOFA score, Glasgow coma score (GCS), site of infection, Combined chronic obstructive pulmonary disease (COPD), blood lactic acid (Lac), prothrombin time (PT), albumin (Alb), total bilirubin (TBil), pH, proportion of mechanical ventilation, duration of mechanical ventilation, proportion of vasoactive medication used, and maximal concentration, length of ICU stay, hospital stay, incidence of acute kidney injury, in-hospital mortality, 30-day mortality among the four groups. Multivariate Cox regression analysis showed that after adjusting for confounding factors, for every 1 cmHg increase in PaO2/FiO2 at ICU admission, the 30-day mortality risk decreased by 2% [hazard ratio (HR) = 0.98, 95% confidence interval (95%CI) was 0.98-0.99, P < 0.001]. The 30-day mortality risk in the Q4 group was reduced compared with the Q1 group by 41% (HR = 0.59, 95%CI was 0.46-0.76, P < 0.001). The fitted curve showed that a curvilinear relationship between PaO2/FiO2 and 30-day mortality after adjustment for confounders. In the inflection point analysis, for every 1 cmHg increase in PaO2/FiO2 at PaO2/FiO2 < 28.55 cmHg, the risk of 30-day death in sepsis patients was reduced by 5% (HR = 0.95, 95%CI was 0.94-0.97, P < 0.001); when PaO2/FiO2 ≥ 28.55 cmHg, there was no statistically significant association between PaO2/FiO2 and the increase in the risk of 30-day death in sepsis (HR = 1.01, 95%CI was 0.99-1.02, P = 0.512). ROC curve analysis showed that the area under the curve (AUC) for the prediction of 30-day mortality by admission PaO2/FiO2 in ICU sepsis patients was 0.650, which was lower than the predictive ability of the SOFA score (AUC = 0.698) and APACHE II score (AUC = 0.723).
CONCLUSION
In patients with sepsis, PaO2/FiO2 at ICU admission is strongly associated with 30-day mortality risk, alerting healthcare professionals to pay attention to patients with low PaO2/FiO2 for timely interventions.
Humans
;
Sepsis/mortality*
;
Intensive Care Units
;
Retrospective Studies
;
Prognosis
;
Hospital Mortality
;
Oxygen
;
Male
;
Predictive Value of Tests
;
Female
;
Middle Aged
;
Aged
5.Role of macrophages in the pathogenesis of septic cardiomyopathy.
Linke ZHANG ; Zhiling ZHAO ; Tingcui LI ; Wen LI ; Yuxin LENG ; Qinggang GE
Chinese Critical Care Medicine 2025;37(3):305-309
Sepsis is a life-threatening organ dysfunction caused by the body's dysregulated response to infection. Reversible myocardial dysfunction caused by sepsis is known as septic cardiomyopathy. A thorough understanding of the pathogenesis of septic cardiomyopathy is crucial for early intervention to prevent its progression and improve the success rate of sepsis treatment. At present, the research on the pathogenesis of septic cardiomyopathy mainly focuses on two aspects: the systemic neuroimmune mechanism and the local changes of cardiomyocytes. The former mainly includes the autonomic nervous dysfunction mainly caused by sympathetic overactivation and the inflammatory storm induced by immune response disorder. The latter covers the dysregulation of calcium homeostasis, mitochondrial dysfunction and energy metabolism disorder of cardiomyocytes. Immune dysfunction is one of the key factors that cause the poor prognosis of patients with septic cardiomyopathy. Macrophages are sentinel cells of the body's innate immunity. Cardiac macrophages have been confirmed to be one of the most heterogeneous immune cells in the heart. According to their origin and differentiation, they can be divided into bone marrow-derived tissue infiltrating macrophages and cardiac resident macrophages, which have roles of polarization, phagocytosis, regulation of inflammatory response, and participate in innate and adaptive immunity. In the occurrence and development of septic cardiomyopathy, cardiac macrophages recruited from the blood participate in balancing the inflammation and repair of myocardial tissue through the conversion of pro-inflammatory phenotype and anti-inflammatory phenotype. Cardiac resident macrophages mediate immune phagocytosis to maintain the local homeostasis of cardiomyocytes, and the glycometabolic reprogramming of macrophages regulates the release of inflammatory factors, while macrophage metabolic reprogramming regulates the release of inflammatory factors. A deeper understanding of the biological behavior of macrophages, and regulating the polarization, metabolism and phagocytosis of cardiac macrophages, could serve as new target for the prevention and treatment of septic cardiomyopathy. Therefore, this article reviews the key pathogenesis of septic cardiomyopathy and the role of macrophages of different origins and differentiation, revealing the possibility of developing new strategies for the prevention and treatment of septic cardiomyopathy.
Humans
;
Cardiomyopathies/pathology*
;
Macrophages/immunology*
;
Sepsis/complications*
;
Myocytes, Cardiac
6.Analysis of the safety of dinutuximab β for the treatment of neuroblastoma
Anle SHEN ; Yali HAN ; Liting YU ; An'an ZHANG ; Jie ZHAO ; Qiushi YANG ; Haonan LI ; Zhiling LI ; Yijin GAO
Journal of Chongqing Medical University 2025;50(8):1042-1046
Objective:To analyze the clinical characteristics of adverse reactions caused by dinutuximab β for the treatment of neuro-blastoma(NB)in China and to provide safety evidence for the rational use of dinutuximab β in clinical practice.Methods:Clinical data were retrospectively collected from 16 pediatric patients with NB who had been treated with dinutuximab β at Shanghai Children's Medical Center Affiliated to Shanghai Jiao Tong University School of Medicine from January 2022 to November 2023,and the adverse reactions caused by dinutuximab β were summarized and analyzed.Results:The male-to-female ratio was 5:3 among the 16 children with NB.The retroperitoneum was the main initial site of involvement,accounting for 75%.Thirteen(81.25%)patients had high-risk NB.The adverse reactions caused by dinutuximab β mainly included decreased hemoglobin,fever,vomiting,and diarrhea.The inci-dence of adverse reactions was highest in the first course of treatment,and the median time of adverse reactions was 2-5 days.Conclu-sion:Targeted monitoring should be carried out at an early stage during dinutuximab β administration.Adverse reactions should be de-tected and managed early to ensure the safety of medication for children.
7.Orthopedic disease spectrum among soldiers in a new type of naval hospital ship during medical patrol at sea
Chengyun GAO ; Xinle HUANG ; Xuya SHANG ; Zhiling ZHANG ; Feng LI
Journal of Navy Medicine 2025;46(3):248-251
Objective To summarize the characteristics of the constitution of orthopedic diseases among soldiers in a sea area,so as to provide the reference for the rational allocation of medical resources,the prevention and treatment of orthopedic diseases,and the improvement of naval medical support.Methods Medical records on injuries of the outpatient and emergency departments in a hospital ship from February 2021 to April 2022 were retrospectively analyzed.The number of varying orthopedic diseases was counted,and descriptive statistical analysis was conducted.Results A total of 200 cases were included in the statistical analysis.The top five diseases were chronic low back pain,knee pain,tibial contusion,wrist joint injury,and shoulder joint injury,and they accounted for 82%of the total orthopedic diseases.Conclusion The medical team stationed on hospital ships should be guided by actual medical needs of officers and soldiers on island,enhance the diagnosis and treatment of common orthopedic diseases,formulate scientific and reasonable prevention and treatment strategies.The allocation of medical materials and personnel should be in line with the spectrum of orthopedic diseases as far as possible to reduce non-combat casualty and strengthen the combat effectiveness of the army.
8.Correlation of KRAS Gene 3'UTR Polymorphisms with Cervical Cancer and Cervical Intraepithelial Neoplasia in Chinese Han Population in Yunnan Province
Ni GUO ; Cheng ZHANG ; Chao HONG ; Weipeng LIU ; Yufeng YAO ; Zhiling YAN
Journal of Kunming Medical University 2024;45(2):14-22
Objective To investigate the correlation between rs712 and rs7973450 located at the 3'UTR region of the KRAS gene and the risk of cervical cancer(CC)and cervical intraepithelial neoplasia(CIN)in Chinese Han population in Yunnan province.Methods A total of 2405 individuals(461 subjects with CIN,961 subjects with CC and 983 healthy controls)were enrolled.The SNPs were genotyped used TaqMan assay and the correlation of these SNPs with CIN and CC was analyzed.Results The A allele of rs7973450 might be a protective factor for the occurrence of CIN(P = 0.004,OR= 0.651,95%CI 0.487~0.871)and CC(P = 7.00×10-4,OR= 0.667,95%CI 0.529~0.844).There was no significant difference in allelic and genotypic distribution of rs712 among CIN,CC and Control groups(P>0.017).The haplotype assay showed thatrs712A-rs7973450G was associated with increased risk of CIN(P = 4.00×10-4;OR= 1.714,95%CI 1.269~2.314)and CC(P = 3.84×10-5,OR= 1.667,95%CI 1.305~2.131).While haplotype rs712A-rs7973450A was associated with a lower risk of CC(P = 0.012,OR= 0.790,95%CI 0.658~0.950).Conclusion The A allele of rs7973450 in 3'UTR of KRAS gene might be the protective factor for the occurrence of CIN and CC in a Chinese Han population in Yunnan province.
9.Construction and validation of a predictive model for early occurrence of lower extremity deep venous thrombosis in ICU patients with sepsis
Zhiling QI ; Detao DING ; Cuihuan WU ; Xiuxia HAN ; Zongqiang LI ; Yan ZHANG ; Qinghe HU ; Cuiping HAO ; Fuguo YANG
Chinese Critical Care Medicine 2024;36(5):471-477
Objective:To investigate the risk factors of lower extremity deep venous thrombosis (LEDVT) in patients with sepsis during hospitalization in intensive care unit (ICU), and to construct a nomogram prediction model of LEDVT in sepsis patients in the ICU based on the critical care scores combined with inflammatory markers, and to validate its effectiveness in early prediction.Methods:726 sepsis patients admitted to the ICU of the Affiliated Hospital of Jining Medical University from January 2015 to December 2021 were retrospectively included as the training set to construct the prediction model. In addition, 213 sepsis patients admitted to the ICU of the Affiliated Hospital of Jining Medical University from January 2022 to June 2023 were retrospectively included as the validation set to verify the performance of the prediction model. Clinical data of patients were collected, such as demographic information, vital signs at the time of admission to the ICU, underlying diseases, past history, various types of scores within 24 hours of admission to the ICU, the first laboratory indexes of admission to the ICU, lower extremity venous ultrasound results, treatment, and prognostic indexes. Lasso regression analysis was used to screen the influencing factors for the occurrence of LEDVT in sepsis patients, and the results of Logistic regression analysis were synthesized to construct a nomogram model. The nomogram model was evaluated by receiver operator characteristic curve (ROC curve), calibration curve, clinical impact curve (CIC) and decision curve analysis (DCA).Results:The incidence of LEDVT after ICU admission was 21.5% (156/726) in the training set of sepsis patients and 21.6% (46/213) in the validation set of sepsis patients. The baseline data of patients in both training and validation sets were comparable. Lasso regression analysis showed that seven independent variables were screened from 67 parameters to be associated with the occurrence of LEDVT in patients with sepsis. Logistic regression analysis showed that the age [odds ratio ( OR) = 1.03, 95% confidence interval (95% CI) was 1.01 to 1.04, P < 0.001], body mass index (BMI: OR = 1.05, 95% CI was 1.01 to 1.09, P = 0.009), venous thromboembolism (VTE) score ( OR = 1.20, 95% CI was 1.11 to 1.29, P < 0.001), activated partial thromboplastin time (APTT: OR = 0.98, 95% CI was 0.97 to 0.99, P = 0.009), D-dimer ( OR = 1.03, 95% CI was 1.01 to 1.04, P < 0.001), skin or soft-tissue infection ( OR = 2.53, 95% CI was 1.29 to 4.98, P = 0.007), and femoral venous cannulation ( OR = 3.72, 95% CI was 2.50 to 5.54, P < 0.001) were the independent influences on the occurrence of LEDVT in patients with sepsis. The nomogram model was constructed by combining the above variables, and the ROC curve analysis showed that the area under the curve (AUC) of the nomogram model for predicting the occurrence of LEDVT in patients with sepsis was 0.793 (95% CI was 0.746 to 0.841), and the AUC in the validation set was 0.844 (95% CI was 0.786 to 0.901). The calibration curve showed that its predicted probability was in good agreement with the actual probabilities were in good agreement, and both CIC and DCA curves suggested a favorable net clinical benefit. Conclusion:The nomogram model based on the critical illness scores combined with inflammatory markers can be used for early prediction of LEDVT in ICU sepsis patients, which helps clinicians to identify the risk factors for LEDVT in sepsis patients earlier, so as to achieve early treatment.
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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