1.Clinical features and prognosis of acute B lymphoblastic leukemia children carrying a TCF3: : PBX1 fusion gene
Lulu HUANG ; Yunyan HE ; Yang LI ; Danna LIN ; Ning LIAO ; Yayun LING ; Lyuhong XU ; Xinyu LI ; Huirong MAI ; Ying WANG ; Wuqing WAN ; Ying LIU ; Yanlai TANG ; Xiaoli ZHANG ; Chuan TIAN ; Xiaofeng LI ; Qiwen CHEN ; Xingjiang LONG ; Liuhua LIAO ; Qiaoru LI ; Jianling CAI ; Zijun ZHEN ; Zhiguang LI ; Keyan YANG ; Qinlong ZHENG ; Lihua YANG
Chinese Journal of Applied Clinical Pediatrics 2025;40(7):497-502
Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.
2.Clinical features and prognosis of acute B lymphoblastic leukemia children carrying a TCF3: : PBX1 fusion gene
Lulu HUANG ; Yunyan HE ; Yang LI ; Danna LIN ; Ning LIAO ; Yayun LING ; Lyuhong XU ; Xinyu LI ; Huirong MAI ; Ying WANG ; Wuqing WAN ; Ying LIU ; Yanlai TANG ; Xiaoli ZHANG ; Chuan TIAN ; Xiaofeng LI ; Qiwen CHEN ; Xingjiang LONG ; Liuhua LIAO ; Qiaoru LI ; Jianling CAI ; Zijun ZHEN ; Zhiguang LI ; Keyan YANG ; Qinlong ZHENG ; Lihua YANG
Chinese Journal of Applied Clinical Pediatrics 2025;40(7):497-502
Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.
3.Construction and evaluation of a nomogram prediction model of atherogenesis risk in patients with type 2 diabetes mellitus
Chaojun SHI ; Zijun LIU ; Yifan WANG ; Weiqin CAI ; Qi JING ; Hongqing AN ; Qianqian GAO
Journal of Public Health and Preventive Medicine 2024;35(5):56-59
Objective To analyze the risk factors influencing the occurrence of atherosclerosis in patients with type 2 diabetes, and to construct and evaluate a nomogram prediction model. Methods Multivariate logistic regression was used to analyze the risk factors of atherosclerosis in type 2 diabetes mellitus, and R software was used to build a nomogram prediction model. The accuracy and clinical validity of the model were verified by using H-L fit curve, area under ROC curve and calibration curve. Results The prevalence rate of atherosclerosis was 56.37%. Independent risk factors for atherosclerosis in type 2 diabetes mellitus (P<0.05) were body weight (OR=1.42,P<0.05), glycated serum protein (OR=1.35, P<0.05), lactate dehydrogenase (OR=1.17, P<0.05), alkaline phosphatase (OR=0.79, P<0.05), hyperlipidemia (OR=2.30, P<0.05), stroke (OR=4.20, P<0.05), coronary heart disease (OR=64.54, P<0.05), lower extremity artery disease (OR=24.52, P<0.05), and other endocrine diseases (OR=1.65 , P<0.05). The area under ROC curve was 0.91, the slope of the calibration curve was close to 1, and the H-L fit curve χ2=3.11. The internal verification result of the constructed nomogram prediction model was P=0.93. External verification of patients in the test set showed that the area under ROC curve was 0.91, indicating good differentiation and accuracy of the model. Conclusion The prediction model established by using the risk factors screened in this study has a high accuracy and differentiation, and medical staff can take effective prevention measures according to the individual factors of patients.
4.A Protocol for Developing Chinese Clinical Practice Guidelines of Hypertension
Ying LOU ; Wenjun MA ; Zijun WANG ; Nan YANG ; Yajia SUN ; Yunlan LIU ; Ruobing LEI ; Junxian ZHAO ; Xufei LUO ; Lu WANG ; Yaolong CHEN ; Yaling HAN ; Yingxian SUN ; Yuming LI ; Jun CAI
Cardiology Discovery 2024;04(3):187-191
To improve the standard screening, diagnosis, and treatment of hypertension in patients in China; realize the standardization of clinical practice of hypertension; and improve the prevention and control level of hypertension in China, it is both important and necessary to develop a clinical practice guideline for hypertension according to a recognized methodology. Jointly sponsored by the National Center for Cardiovascular Diseases, Chinese Medical Doctor Association, Hypertension Committee of the Chinese Medical Doctor Association, Chinese Society of Cardiology, and Hypertension Committee of Cross-Straits Medicine Exchange Association, the "Chinese Clinical Practice Guidelines of Hypertension" was proposed. Research Unit of Evidence-Based Evaluation and Guidelines, Chinese Academy of Medical Sciences, Guideline and Standards Research Centre of Chinese Medical Association Publishing House, Lanzhou University Institute of Health Data Science, and Lanzhou University GRADE Center will provide methodological support for the guidelines.
5.A Protocol for Developing Chinese Clinical Practice Guidelines of Hypertension
Ying LOU ; Wenjun MA ; Zijun WANG ; Nan YANG ; Yajia SUN ; Yunlan LIU ; Ruobing LEI ; Junxian ZHAO ; Xufei LUO ; Lu WANG ; Yaolong CHEN ; Yaling HAN ; Yingxian SUN ; Yuming LI ; Jun CAI
Cardiology Discovery 2024;04(3):187-191
To improve the standard screening, diagnosis, and treatment of hypertension in patients in China; realize the standardization of clinical practice of hypertension; and improve the prevention and control level of hypertension in China, it is both important and necessary to develop a clinical practice guideline for hypertension according to a recognized methodology. Jointly sponsored by the National Center for Cardiovascular Diseases, Chinese Medical Doctor Association, Hypertension Committee of the Chinese Medical Doctor Association, Chinese Society of Cardiology, and Hypertension Committee of Cross-Straits Medicine Exchange Association, the "Chinese Clinical Practice Guidelines of Hypertension" was proposed. Research Unit of Evidence-Based Evaluation and Guidelines, Chinese Academy of Medical Sciences, Guideline and Standards Research Centre of Chinese Medical Association Publishing House, Lanzhou University Institute of Health Data Science, and Lanzhou University GRADE Center will provide methodological support for the guidelines.
6.Safety study of naxitamab infusion for neuroblastoma under modified conditioning re-gimen
Zhu JIA ; Deng JUN ; Que YI ; Liu LONGZHEN ; Wu LIUHONG ; Yu WANQI ; Guan HUIJIE ; Wang JUAN ; Lu SUYING ; Sun FEIFEI ; Huang JUNTING ; Song MENGJIA ; Zhen ZIJUN ; Cai RUIQING ; Zhang YIZHUO
Chinese Journal of Clinical Oncology 2024;51(22):1154-1158
Objective:Glucorticoid therapy has the potential to mitigate immunogical effect of naxitamab. Ketamine is an anesthetic medica-tion and cause weak or shallow breathing. This article is to analyze the effect of modified conditioning regimen with substitution re-mifentanil for ketamine and without glucorticoid therapy on adverse events associated with naxitamab. Methods:Clinical data with naxit-amab infusion under modified conditioning regimen in Sun Yat-sen University Cancer Center between June 2023 and June 2024 were re-trieved to analyze adverse events and risk factors. Results:Overall,seventeen patients underwent 201 infusions. The most frequent adverse events were as follows:neurological pain (all grades) 93.0%,hypertension 55.7%,hypotension 34.8%,respectively. Bronchospasm and hyp-oxia were seen in 3.0% and 10.9% infusions,respectively. Fever occurred less frequently in the second cycle of infusion. No patients suspen-ded infusion due to severe adverse event. Conclusions:The infusion of naxitamab is tolerable under the modified conditioning regimen and adverse event is less than expected and controllable.
7.Safety study of naxitamab infusion for neuroblastoma under modified conditioning re-gimen
Zhu JIA ; Deng JUN ; Que YI ; Liu LONGZHEN ; Wu LIUHONG ; Yu WANQI ; Guan HUIJIE ; Wang JUAN ; Lu SUYING ; Sun FEIFEI ; Huang JUNTING ; Song MENGJIA ; Zhen ZIJUN ; Cai RUIQING ; Zhang YIZHUO
Chinese Journal of Clinical Oncology 2024;51(22):1154-1158
Objective:Glucorticoid therapy has the potential to mitigate immunogical effect of naxitamab. Ketamine is an anesthetic medica-tion and cause weak or shallow breathing. This article is to analyze the effect of modified conditioning regimen with substitution re-mifentanil for ketamine and without glucorticoid therapy on adverse events associated with naxitamab. Methods:Clinical data with naxit-amab infusion under modified conditioning regimen in Sun Yat-sen University Cancer Center between June 2023 and June 2024 were re-trieved to analyze adverse events and risk factors. Results:Overall,seventeen patients underwent 201 infusions. The most frequent adverse events were as follows:neurological pain (all grades) 93.0%,hypertension 55.7%,hypotension 34.8%,respectively. Bronchospasm and hyp-oxia were seen in 3.0% and 10.9% infusions,respectively. Fever occurred less frequently in the second cycle of infusion. No patients suspen-ded infusion due to severe adverse event. Conclusions:The infusion of naxitamab is tolerable under the modified conditioning regimen and adverse event is less than expected and controllable.
9.Effects of preoperative nutritional status on postoperative functional prognosis in elderly patients with proximal humerus fracture
Xin JIAO ; Kun ZHANG ; Yangjun ZHU ; Zhe SONG ; Yuewen NIAN ; Xiao CAI ; Jun ZHANG ; Lisong HENG ; Zijun LI
Chinese Journal of Orthopaedic Trauma 2022;24(8):673-678
Objective:To investigate the effects of preoperative nutritional status on postoperative functional prognosis in elderly patients with proximal humerus fracture.Methods:From January 2020 to December 2020, 103 elderly patients (≥65 years old) were treated for proximal humerus fractures by open reduction and internal fixation at Department of Traumatology, Honghui Hospital Affiliated to Xi'an Jiaotong University. Upon admission, according to the Geriatric Nutrition Risk Index (GNRI), they were assigned into a normal nutrition group (55 cases, with GNRI≥92) and a malnutrition group (48 cases, with GNRI<92). The baseline data, preoperative hemoglobin level, time from injury to operation, intraoperative blood transfusion, postoperative complications, 1-year mortality, and Neer shoulder functional scores at 3 months, 6 months and the last follow-up were compared between the 2 groups.Results:The 2 groups were comparable because there were no significant differences in gender, injury side, Neer fracture classification, injury cause, or American Society of Anesthesiologists (ASA) grading ( P>0.05). The age of the malnutrition group was significant older than that of the normal nutrition group ( P<0.05). All patients were followed up for 9 to 16 months (mean, 13.6 months) after surgery. In the normal nutrition group and the malnutrition group, respectively, the preoperative hemoglobin level was (10.24±0.68) g/dL and (8.94±0.89) g/dL, the time from injury to operation (3.9±1.3) d and (5.8±1.2) d, the rate of intraoperative blood transfusion 14.5%(8/55) and 60.4%(29/48), the rate of postoperative complications 20.0%(11/55) and 39.6%(19/48), the 1-year mortality 1.8%(1/55)、4.2%(2/48), and the Neer shoulder function score (46.7±8.8) points and (43.2±5.6) points at 3 months after operation, (67.6±6.2) points and (76.3±5.5) points at 6 months after operation, and (80.4±5.0) points and (76.3±5.5) points at the last follow-up. Comparisons of all the above items showed significant differences between the 2 groups (all P<0.05). Conclusions:Preoperative malnutrition in elderly patients with proximal humerus fracture has adverse effects on preoperative waiting time, intraoperative blood transfusion, complications and postoperative shoulder function. Therefore, perioperatively, attention should be paid to the nutritional status of elderly patients to reduce their stress responses to fracture, surgery and anesthesia, and to improve their postoperative function and quality of life.
10.Analysis of NF1 gene mutations in two sporadic patients with neurofibromatosis type 1.
Xiaoyan ZHAO ; Qian ZHOU ; Liangqi CAI ; Zijun ZHAO ; Linglin ZHANG ; Peiru WANG ; Guolong ZHANG
Chinese Journal of Medical Genetics 2018;35(4):489-492
OBJECTIVETo detect mutations of the NF1 gene in two sporadic cases with neurofibromatosis type 1 (NF1) and explore their molecular mechanisms.
METHODSClinical data of the two patients was collected. Genomic DNA was extracted from peripheral blood samples. Specific primers were designed to exclude pseudogenes. PCR was performed to amplify all coding exons of the NF1 gene. PCR products were directly sequenced.
RESULTSTwo novel mutations of the NF1 gene (c.1019-1020delCT in exon 9 and c.7189G to A in exon 48) were respectively identified in the two patients but not among their unaffected parents or 100 healthy controls.
CONCLUSIONMutations of the NF1 gene may have predisposed to the NF1 in the two patients.


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