Objective:To explore the clinical features and molecular characteristics of myeloproliferative neoplasms (MPNs) patients with NFE2 gene mutations.Methods:Gene targeted sequencing was used to detect NFE2 gene mutation in 723 patients diagnosed with MPNs who were admitted to Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College between April 2021 and June 2023. The association between NFE2 gene mutations and clinical features and molecular characteristics of MPNs patients were retrospectively analyzed.Results:Among 723 patients with MPNs, NFE2 gene mutations were found in 41 cases (5.7%) . NFE2 gene mutations were predominantly frameshift mutations (44.4%) , followed by nonsense mutations (33.3%) . The median number of mutations in patients with NFE2 gene mutations (4 [2,5]) was higher compared to the group without NFE2 gene mutations (2, [1,3]) ( P<0.001) . NFE2 gene mutations frequently co-occurred with mutations in MPL, ATM, PPM1D, and TET1. NFE2 gene mutations were mostly sub-clonal events, with 80.5% occurring after MPNs driver mutations (JAK2, CALR, or MPL) . NFE2 mutations were correlated with older age [median age: 60 (54, 67) years vs 54 (41, 63) years, P=0.001]. Patients with NFE2 gene mutations had a higher incidence of pre-diagnosis thrombosis (39.0% vs 22.0%, P=0.012) and pre-diagnosis arterial thrombosis (36.6% vs 20.4%, P=0.014) . Using a logistic regression analysis model adjusting for age and comorbidities (including chronic infections, malignancies, and autoimmune diseases) , NFE2 gene mutation was identified as an independent determinant of elevated tumor necrosis factor-alpha (TNF-α) ( OR=2.747, 95% CI: 1.143-6.605, P=0.024) , interferon-gamma (IFN-γ) ( OR=2.689, 95% CI: 1.191-6.076, P=0.017) , IL-10 ( OR=3.219, 95% CI: 1.343-7.717, P=0.009) , IL-12P70 ( OR=3.397, 95% CI:1.003-11.508, P=0.049) , IL-17 ( OR=2.284, 95% CI: 1.017-5.127, P=0.045) . In polycythaemia vera (PV) patients with the NFE2 gene mutation, the proportion of those classified as high-risk is notably higher in both the IWG-PV and mutation-enhanced international prognostic systems for PV (MIPSS-PV) (66.7% vs 25.3% for IWG-PV, P=0.033; 22.2% vs 2.0% for MIPSS-PV, P=0.013) . Similarly, for essential thrombocythaemia (ET) patients, the proportion in the high-risk group of the mutation-enhanced international prognostic systems for ET (MIPSS-ET) is significantly higher (15.4% vs 6.1%, P=0.021) . No statistically significant differences were observed in overall survival or cumulative incidence of thrombosis between NFE2-mutated (38 cases) and non-mutated MPNs patients (671 cases, P>0.05) . Conclusion:NFE2 gene mutations in MPNs were predominantly frameshift mutations. NFE2 gene mutations were correlated with older age, elevated levels of several inflammatory factors (including TNF-α、IFN-γ、IL-10、IL-12P70、IL-17) , and they mostly occurred in late-stage of MPNs.

Objective:To explore the clinical features and molecular characteristics of myeloproliferative neoplasms (MPNs) patients with NFE2 gene mutations.Methods:Gene targeted sequencing was used to detect NFE2 gene mutation in 723 patients diagnosed with MPNs who were admitted to Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College between April 2021 and June 2023. The association between NFE2 gene mutations and clinical features and molecular characteristics of MPNs patients were retrospectively analyzed.Results:Among 723 patients with MPNs, NFE2 gene mutations were found in 41 cases (5.7%) . NFE2 gene mutations were predominantly frameshift mutations (44.4%) , followed by nonsense mutations (33.3%) . The median number of mutations in patients with NFE2 gene mutations (4 [2,5]) was higher compared to the group without NFE2 gene mutations (2, [1,3]) ( P<0.001) . NFE2 gene mutations frequently co-occurred with mutations in MPL, ATM, PPM1D, and TET1. NFE2 gene mutations were mostly sub-clonal events, with 80.5% occurring after MPNs driver mutations (JAK2, CALR, or MPL) . NFE2 mutations were correlated with older age [median age: 60 (54, 67) years vs 54 (41, 63) years, P=0.001]. Patients with NFE2 gene mutations had a higher incidence of pre-diagnosis thrombosis (39.0% vs 22.0%, P=0.012) and pre-diagnosis arterial thrombosis (36.6% vs 20.4%, P=0.014) . Using a logistic regression analysis model adjusting for age and comorbidities (including chronic infections, malignancies, and autoimmune diseases) , NFE2 gene mutation was identified as an independent determinant of elevated tumor necrosis factor-alpha (TNF-α) ( OR=2.747, 95% CI: 1.143-6.605, P=0.024) , interferon-gamma (IFN-γ) ( OR=2.689, 95% CI: 1.191-6.076, P=0.017) , IL-10 ( OR=3.219, 95% CI: 1.343-7.717, P=0.009) , IL-12P70 ( OR=3.397, 95% CI:1.003-11.508, P=0.049) , IL-17 ( OR=2.284, 95% CI: 1.017-5.127, P=0.045) . In polycythaemia vera (PV) patients with the NFE2 gene mutation, the proportion of those classified as high-risk is notably higher in both the IWG-PV and mutation-enhanced international prognostic systems for PV (MIPSS-PV) (66.7% vs 25.3% for IWG-PV, P=0.033; 22.2% vs 2.0% for MIPSS-PV, P=0.013) . Similarly, for essential thrombocythaemia (ET) patients, the proportion in the high-risk group of the mutation-enhanced international prognostic systems for ET (MIPSS-ET) is significantly higher (15.4% vs 6.1%, P=0.021) . No statistically significant differences were observed in overall survival or cumulative incidence of thrombosis between NFE2-mutated (38 cases) and non-mutated MPNs patients (671 cases, P>0.05) . Conclusion:NFE2 gene mutations in MPNs were predominantly frameshift mutations. NFE2 gene mutations were correlated with older age, elevated levels of several inflammatory factors (including TNF-α、IFN-γ、IL-10、IL-12P70、IL-17) , and they mostly occurred in late-stage of MPNs.

To date,the information technology develops rapidly and the dawn of digital era comes just around the cor-ner.Under these new situations,smart Party building management platforms stand as a new impetus,energizing the Party build-ing efforts with robust capabilities for data storage,analysis,automatic computation,and administration.These platforms provide comprehensive data support that enhances the precision and efficiency of Party building.This leads to an invigorated process of continuous innovation and high-quality development within the Party building.This article delves into the practical application of the current unit's smart party building management platform,examining the challenges encountered during its operation.It also posits several reflections and strategic responses aimed at elevating the scientific management standards of party building.

Rhamnogalacturonan Ⅱ(RG-Ⅱ)is one of the structural domains of pectin whose structure is highly conserved among species.The main chain of RG-Ⅱ consists of approximately nine galacturonic acids linked by α-1,4 glycosidic bonds,with six well-defined side chains replacing them(A-F).The structures of the disaccharide side chains C and D and the monosaccharide side chains E and F in RG-Ⅱ from different sources remain essentially the same.In contrast,the oligosaccharide side chains A and B showed slight variability.Structural characterization of RG-Ⅱ can be achieved by molecular weight,monosaccharide composition,and mass spectrometry.The polysaccharides containing RG-Ⅱ structural domains in traditional Chinese medicines(TCMs)have high medicinal value.Isolation of RG-Ⅱ can be achieved using endo-polygalacturonase(Endo-PG)and Penicillium oxalicum.A substantial number of RG-Ⅱ standards can be rapidly prepared from red wine for the development of new quantitative methods to realize the quality control of active polysaccharides from traditional Chinese medicines and to promote the research process of polysaccharides from traditional Chinese medicines.

Humans ; Infant, Newborn ; Intensive Care Units, Neonatal ; Logistic Models ; Phenotype ; Risk Factors

Objective:This study aimed to explore the efficacy and safety of rituximab combined with short-course and intensive regimens in the treatment of adult patients with Burkitt leukemia.Methods:The clinical data of 11 Burkitt leukemia patients in our hospital from January 30, 2006, to September 12, 2018, were collected. The clinical details, complete remission (CR) rate, overall survival (OS) , relapse-free survival (RFS) , and adverse events were evaluated.Results:The median age of 11 patients was 34 (15-54) years, of which six were males and five were females (M∶F, 1.2∶1) . The median white blood cell (WBC) count was 12.28 (2.21-48.46) ×10 9/L, and the median blast percent of peripheral blood and bone marrow were 40% (3%-76%) and 84.0% (29.5%-94.5%) , respectively. Ten patients were administered with rituximab combined with a short-course and intensive regimens, and two patients underwent autologous hematopoietic stem cell transplantation following consolidation chemotherapy. The CR rate after one cycle of induction therapy was 100%, the four-year OS was 90%, and RFS was 90%. Out of the ten treated patients, only one patient suffered from tumor lysis syndrome during the induction chemotherapy. Consequently, renal function recovered after hemodialysis and other treatments. The regimen is safe with no treatment-related deaths. Conclusions:Rituximab combined with short-course and intensive chemotherapy regimens is effective and well-tolerated in adult Burkitt leukemia.

Objective:To explore the effects of standardized enteral nutrition process in critically ill patients.Methods:We retrieved PubMed, EMBASE, MEDLINE, WanFang Data, China National Knowledge Infrastructure (CNKI) and China Biology Medicine (CBM) by computer to collect literatures on effects of standardized enteral nutrition process in critically ill patients from the establishment of database to May 31, 2019. RevMan 5.3 was used to statistical analysis.Results:Finally, 11 articles were included. Meta-analysis results showed that compared with conventional care, the standardized enteral nutrition process could increase the calorie compliance rate of critical ill patients on the seventh day [ OR=8.18, 95% CI (4.91, 11.45) , P<0.01], and reduce the incidence of feeding intolerance symptoms [ OR=0.36, 95% CI (0.23, 0.57) , P<0.01]. However, the standardized enteral nutrition process did not show obvious advantages in shortening mechanical ventilation, Intensive Care Unit (ICU) hospitalization time and reducing mortality, combined effects, the combined effect was not statistically significant ( P>0.05) . Conclusions:The standardized enteral nutrition process could effectively increase the calorie compliance rate of critical ill patients on the seventh day and reduce the incidence of feeding intolerance symptoms; however, it cannot significantly shorten the time of mechanical ventilation, ICU hospitalization time and reduce the mortality. Further research is needed for verification.

OBJECTIVE: To investigate the effect of dexmedetomidine combined with pulmonary protective ventilation against lung injury in patients undergoing surgeries for esophageal cancer with one-lung ventilation (OLV). METHODS: Forty patients with undergoing surgery for esophageal cancer with OLV were randomly divided into pulmonary protective ventilation strategy group (F group) and dexmedetomidine combined with protective ventilation strategy group (DF group; =20). In F group, lung protective ventilation strategy during anesthesia was adopte, and in DF group, the patients received intravenous infusion of dexmedetomidine hydrochloride (0.3 μg · kg ·h) during the surgery starting at 10 min before anesthesia induction in addition to protective ventilation strategy. Brachial artery blood was sampled before ventilation (T), at 30 and 90 min after the start of OLV (T and T, respectively) and at the end of the surgery (T) for analysis of superoxide dismutase (SOD), malondialdehyde (MDA), tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), arterial oxygenation pressure (PaO), oxygenation index (OI) and lung compliance (CL). RESULTS: At the time points of T, T and T, SOD level was significantly higher and IL-6 level was significantly lower in the DF group than in F group ( < 0.05). The patients in DF group showed significantly higher PaO, OI and CL index than those in F group at all the 3 time points. CONCLUSIONS Dexmedetomidine combined with pulmonary protective ventilation strategy can reduce perioperative lung injury in patients undergoing surgery for esophageal cancer with OLV by suppressing inflammation and oxidative stress to improve lung function and reduce adverse effects of the surgery.
Analgesics, Non-Narcotic ; pharmacology ; therapeutic use ; Dexmedetomidine ; pharmacology ; therapeutic use ; Esophageal Neoplasms ; drug therapy ; surgery ; Humans ; Lung ; drug effects ; surgery ; One-Lung Ventilation ; Oxidative Stress ; drug effects ; Treatment Outcome

OBJECTIVE: To investigate the effect of dexmedetomidine combined with pulmonary protective ventilation against lung injury in patients undergoing surgeries for esophageal cancer with one-lung ventilation (OLV). METHODS: Forty patients with undergoing surgery for esophageal cancer with OLV were randomly divided into pulmonary protective ventilation strategy group (F group) and dexmedetomidine combined with protective ventilation strategy group (DF group; =20). In F group, lung protective ventilation strategy during anesthesia was adopte, and in DF group, the patients received intravenous infusion of dexmedetomidine hydrochloride (0.3 μg · kg ·h) during the surgery starting at 10 min before anesthesia induction in addition to protective ventilation strategy. Brachial artery blood was sampled before ventilation (T), at 30 and 90 min after the start of OLV (T and T, respectively) and at the end of the surgery (T) for analysis of superoxide dismutase (SOD), malondialdehyde (MDA), tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), arterial oxygenation pressure (PaO), oxygenation index (OI) and lung compliance (CL). RESULTS: At the time points of T, T and T, SOD level was significantly higher and IL-6 level was significantly lower in the DF group than in F group ( < 0.05). The patients in DF group showed significantly higher PaO, OI and CL index than those in F group at all the 3 time points. CONCLUSIONS Dexmedetomidine combined with pulmonary protective ventilation strategy can reduce perioperative lung injury in patients undergoing surgery for esophageal cancer with OLV by suppressing inflammation and oxidative stress to improve lung function and reduce adverse effects of the surgery.
Dexmedetomidine ; Esophageal Neoplasms ; therapy ; Humans ; Lung ; Malondialdehyde ; One-Lung Ventilation

Objective: To investigate the application values of immunophenotypic analysis and molecular genetics in the diagnosis of acute promyelocytic leukemia (APL) . Methods: The retrospective analyses of flow cytometric (FCM) immunophenotypic anyalysis, chromosome karyotype and chromosome fluorescence in situ hybridization (FISH) of 798 outpatient or hospitalization APL patients referred to our hospital between May 2012 and December 2017 were performed to further study the application values of FCM and molecular genetics in the diagnosis of APL. Results: The sensitivity and specificity of FCM were 91.9% and 98.7% respectively. The typical characteristic immunophenotype for APL was as of follows: a high SSC, absence of expression of cluster differntiation (CD) CD34 and HLA-DR, and expression or stronger expression of CD33, consistent expression of CD13, CD9, CD123, expression of CD56, CD7, CD2 (sometimes) . The rest 10% of the cases harbored atypical APL phenotypes, generally accompanied by CD34 and/or HLA-DR expression, decreased SSC and often accompanied by CD2 expression, it was difficult to definitively diagnose APL by this FCM phenotype, and their diagnoses depended on the results of genetics or molecular biology tests. Compared with normal individuals, complex karyotypes APL with t (15;17) translocation, other variant translocations and variant t (11;17) , t (5;17) had no significant differences in terms of their FCM phenotypes. Conclusions FCM could rapidly and effectively diagnose APL. Despite the fact that complex karyotypes with various additional chromosomal abnormalities were detected in approximately one third of APL cases in addition to the pathognomonic t (15;17) (q22;q21) , they had no observable impact on the overall immunophenotype. Molecular and genetic criteria were the golden criteria for the diagnosis of APL. About 10% of immunophenotyping cases relied on molecular genetics for diagnosis.