1.XK gene deletion leading to McLeod syndrome and high-frequency antigen antibodies: identification and transfusion strategy
Jing LI ; Kewen YAO ; Yun DU ; Haiyan HU ; Hongli ZHANG
Chinese Journal of Blood Transfusion 2025;38(8):1107-1112
		                        		
		                        			
		                        			Objective: To investigate the hematological characteristics of the rare McLeod phenotype associated with X-linked chronic granulomatous disease, KEL and XK gene analysis, identification of unexpected antibodies, serological characteristics of high-frequency antigen antibodies, and transfusion strategies. Methods: Serological methods were employed to determine the ABO, Rh, and other blood group system antigen phenotypes of the child, along with screening and identification of unexpected antibodies. The titers of high-frequency antigen antibodies were measured using tube antihuman globulin and microcolumn gel card techniques. Kell blood group typing was performed using serological and genotyping methods, while XK gene sequencing was conducted via next-generation sequencing. Peripheral blood smears from the child's mother were examined for erythrocyte morphology. Results: The child's serological results were as follows: blood group O, ccDEE, MM, Le(a-b+), JK(a+b+), Fy(a+b-), and Kell phenotype K-k+, Kp(a-b+). Plasma analysis revealed alloantibodies anti-C、e, as well as a high-frequency antigen antibody anti-KL, with titers of 512 (tube method) and 2 048 (microcolumn gel method). Genotyping results showed KEL genotype K-k+, Kp(a-b+), Js(a-b+), while XK gene NGS identified a hemizygous deletion of exons 1-3 (XK
       N. 01), consistent with XK: -1 or Kx-(McLeod). The mother's peripheral blood smear exhibited prominent acanthocytes. Conclusion: The hematological features of this rare McLeod phenotype with X-CGD include weakened Kell antigen expression and a complete exon deletion in the XK gene. Early clinical attention should be given to the symptoms and laboratory diagnosis of X-linked chronic granulomatous disease in pediatric patients. XK genotyping for McLeod phenotype should be prioritized to guide cautious transfusion strategies, preventing life-threatening complications due to incompatible blood products.
    
		                        		
		                        		
		                        		
		                        	
2.Stability study of umbilical cord mesenchymal stem cells formulation in large-scale production
Wang-long CHU ; Tong-jing LI ; Yan SHANGGUAN ; Fang-tao HE ; Jian-fu WU ; Xiu-ping ZENG ; Tao GUO ; Qing-fang WANG ; Fen ZHANG ; Zhen-zhong ZHONG ; Xiao LIANG ; Jun-yuan HU ; Mu-yun LIU
Acta Pharmaceutica Sinica 2024;59(3):743-750
		                        		
		                        			
		                        			 Umbilical cord mesenchymal stem cells (UC-MSCs) have been widely used in regenerative medicine, but there is limited research on the stability of UC-MSCs formulation during production. This study aims to assess the stability of the cell stock solution and intermediate product throughout the production process, as well as the final product following reconstitution, in order to offer guidance for the manufacturing process and serve as a reference for formulation reconstitution methods. Three batches of cell formulation were produced and stored under low temperature (2-8 ℃) and room temperature (20-26 ℃) during cell stock solution and intermediate product stages. The storage time intervals for cell stock solution were 0, 2, 4, and 6 h, while for intermediate products, the intervals were 0, 1, 2, and 3 h. The evaluation items included visual inspection, viable cell concentration, cell viability, cell surface markers, lymphocyte proliferation inhibition rate, and sterility. Additionally, dilution and culture stability studies were performed after reconstitution of the cell product. The reconstitution diluents included 0.9% sodium chloride injection, 0.9% sodium chloride injection + 1% human serum albumin, and 0.9% sodium chloride injection + 2% human serum albumin, with dilution ratios of 10-fold and 40-fold. The storage time intervals after dilution were 0, 1, 2, 3, and 4 h. The reconstitution culture media included DMEM medium, DMEM + 2% platelet lysate, 0.9% sodium chloride injection, and 0.9% sodium chloride injection + 1% human serum albumin, and the culture duration was 24 h. The evaluation items were viable cell concentration and cell viability. The results showed that the cell stock solution remained stable for up to 6 h under both low temperature (2-8 ℃) and room temperature (20-26 ℃) conditions, while the intermediate product remained stable for up to 3 h under the same conditions. After formulation reconstitution, using sodium chloride injection diluted with 1% or 2% human serum albumin maintained a viability of over 80% within 4 h. It was observed that different dilution factors had an impact on cell viability. After formulation reconstitution, cultivation in medium with 2% platelet lysate resulted in a cell viability of over 80% after 24 h. In conclusion, the stability of cell stock solution within 6 h and intermediate product within 3 h meets the requirements. The addition of 1% or 2% human serum albumin in the reconstitution diluent can better protect the post-reconstitution cell viability. 
		                        		
		                        		
		                        		
		                        	
		                				3.A new pyrazine from Hypecoum erectum  L. 
		                			
		                			Yun LIU ; Meng-ya HU ; Wen-jing ZHANG ; Yu-xin FAN ; Rui-wen XU ; Deng-hui ZHU ; Yan-jun SUN ; Wei-sheng FENG ; Hui CHEN
Acta Pharmaceutica Sinica 2024;59(1):183-187
		                        		
		                        			
		                        			 Four pyrazines were isolated from the 
		                        		
		                        	
4.COVID-19 related autoimmune myopathy: 5 cases report
Mengting YANG ; Yawen ZHAO ; Yikang WANG ; Jingchu YUAN ; Jianwen DENG ; Jing LIU ; Yiming ZHENG ; Wei ZHANG ; Zhaoxia WANG ; Yun YUAN
Chinese Journal of Neurology 2024;57(1):40-46
		                        		
		                        			
		                        			Objective:To analyze the clinical characteristics, imaging, myopathology and outcomes of patients with COVID-19 related autoimmune myopathy.Methods:The clinical features, serum creatine kinase (CK), myositis antibodies, muscle magnetic resonance imaging, myopathology and therapy of 5 patients with COVID-19 related autoimmune myopathy diagnosed in Peking University First Hospital from December 2022 to April 2023 were collected. The effects of the therapy after a short term follow up were analyzed.Results:Among the 5 patients, there were 3 males and 2 females, with onset age of 42-86 years. All patients presented with proximal muscle weakness in the recovery term of COVID-19. Myalgia was noted in 3 cases, dysphagia in 1, skin damage in 2, interstitial lung disease in 1. The serum CK of the 5 patients was 1 663-16 000 IU/L, 1 patient had anti-3-hydroxy-3-methylglutaryl-CoA reductase autoantibodies and 1 patient had anti-signal recognition particle autoantibodies. The electromyography showed myogenic lesions in all patients. Muscle magnetic resonance imaging showed diffuse muscle edema in all patients, myofascial edema in 3 and subcutaneous-tissue edema in 3. The muscle biopsies in 4 patients revealed necrotic myopathy,with high P62 expression in muscle fibers. The electromicroscopy of 2 patients revealed vacuolated mitochondria and intranuclear tubulofilamentous inclusions in muscle fibers. Four patients were treated with glucocorticoids, of whom 2 patients combined with intravenous immunoglobulin, tacrolimus or cyclophosphamide. One case had close monitoring without drug therapy. They showed significant improvement, but the CK was still abnormal in 4 patients.Conclusions:COVID-19 leads to immune mediated myopathy. The manifestation of patients is characterized by proximal predominant weakness and high creatine kinase level. Muscle magnetic resonance imaging shows diffuse muscle edema. The muscle biopsies reveal necrotic myopathy. The effectiveness of immunosuppression needs to be further studied.
		                        		
		                        		
		                        		
		                        	
5.Clinical value of thrombospondin-1 and transforming growth actor-β in predicting post hepatectomy liver failure
Hasimu HAXIAOBIEKE ; Jing WU ; Gang YAO ; Apaer SHADIKE ; Fulati XIAPUKAITI ; Yun-Fei ZHANG ; Tuersunmaimaiti ABUDUSHALAMUU ; Tuxun TUERHONGJIANG
Chinese Journal of Current Advances in General Surgery 2024;27(6):442-446
		                        		
		                        			
		                        			Objective:To explore the dynamic changes of thrombosbondin-1(TSP-1)and transforming growth factor beta(TGF-β)in patients who underwent hepatectomy and their clinical value in predicting post-hepatectomy liver failure(PHLF).Methods:Clinical data of 113 patients who received hepatectomy in the First Affiliated Hospital of Xinjiang Medical University from Janu-ary 2016 to March 2019 were retrospectively analyzed.The cohort comprised 59 males and 54 fe-males,with an average age of(41.00±13.98)years.According to the standard of the International Study Group of Liver Surgery(ISGLS),TSP-1 was divided into the PHLF group(n=40)and the non-PHLF group(n=73).The dynamic changes of plasma TSP-1 and TGF-β plasma levels during peri-operative period were observed.The definition and diagnostic criteria suggested by ISGLS was used to evaluate PHLF.The value of plasma TSP-1 and TGF-β level in predicting PHLF were ana-lyzed by calculating areas under the receiver operating characteristic curves(AUCs).Patients were divided into TSP-1 high group and TSP-1 low groups based upon the ROC cutoff values and their correlation with PHLF was analyzed.T test and Mann-Whitney rank sum test were performed to compare the TSP-1 and TGF-β levels between the two groups.The rate comparison was carried out by Chi-square test or Fisher's exact test.Results:In this study,40 patients experienced vary-ing degrees of PHLF.The plasma levels of TSP-1 on postoperative day I(POD1)and day 7(POD 7)in the PHLF group were significantly higher than those in the non-PHLF group(P<0.05,P<0.01,re-spectively).The area under the ROC curve of plasma TSP-1 level on postoperative 1 d in the diag-nosis of PHLF was 0.725,with sensitivity of 0.864 and specificity of 0.647.The area under the ROC curve of plasma TSP-1 level on postoperative 7 d in the diagnosis of PHLF was 0.81,with sensitiv-ity of 0.818 and specificity of 0.765.Conclusion:The incidence of PHLF is related to the extent of liver resection,Child-Pugh grade and TSP-1 levels on postoperative day 1.The concentration level of TSP-1 on POD land 7 might be utilized as an effective marker for predicting PHLF.
		                        		
		                        		
		                        		
		                        	
6.Progress of interruption of schistosomiasis transmission and prospects in Yunnan Province
Yun ZHANG ; Lifang WANG ; Xiguang FENG ; Mingshou WU ; Meifen SHEN ; Hua JIANG ; Jing SONG ; Jiayu SUN ; Chunqiong CHEN ; Jiaqi YAN ; Zongya ZHANG ; Jihua ZHOU ; Yi DONG ; Chunhong DU
Chinese Journal of Schistosomiasis Control 2024;36(4):422-427
		                        		
		                        			
		                        			 Schistosomiasis was once hyper-endemic in Yunnan Province. Following concerted efforts for over 70 years, remarkable achievements have been made for schistosomiasis control in the province. In 2004, the Mid- and Long-term Plan for Schistosomiasis Prevention and Control in Yunnan Province was initiated in Yunnan Province, and the target for transmission control of schistosomiasis was achieved in the province in 2009. Following the subsequent implementation of the Outline for Key Projects in Integrated Schistosomiasis Control Program (2009—2015) and the 13th Five - year Plan for Schistosomiasis Control in Yunnan Province, no acute schistosomiasis had been identified in Yunnan Province for successive 12 years, and no local Schistosoma japonicum infections had been detected in humans, animals or Oncomelania hupensis snails for successive 6 years in the province by the end of 2020. The transmission of schistosomiasis was interrupted in Yunnan Province in 2020. This review summarizes the history of schistosomiasis, changes in schistosomiasis prevalence and progress of schistosomiasis control in Yunnan Province, and proposes the future priorities for schistosomiasis control in the province. 
		                        		
		                        		
		                        		
		                        	
7.Species-level Microbiota of Biting Midges and Ticks from Poyang Lake
Jian GONG ; Fei Fei WANG ; Qing Yang LIU ; Ji PU ; Zhi Ling DONG ; Hui Si ZHANG ; Zhou Zhen HUANG ; Yuan Yu HUANG ; Ben Ya LI ; Xin Cai YANG ; Meihui Yuan TAO ; Jun Li ZHAO ; Dong JIN ; Yun Li LIU ; Jing YANG ; Shan LU
Biomedical and Environmental Sciences 2024;37(3):266-277,中插1-中插3
		                        		
		                        			
		                        			Objective The purpose of this study was to investigate the bacterial communities of biting midges and ticks collected from three sites in the Poyang Lake area,namely,Qunlu Practice Base,Peach Blossom Garden,and Huangtong Animal Husbandry,and whether vectors carry any bacterial pathogens that may cause diseases to humans,to provide scientific basis for prospective pathogen discovery and disease prevention and control. Methods Using a metataxonomics approach in concert with full-length 16S rRNA gene sequencing and operational phylogenetic unit(OPU)analysis,we characterized the species-level microbial community structure of two important vector species,biting midges and ticks,including 33 arthropod samples comprising 3,885 individuals,collected around Poyang Lake. Results A total of 662 OPUs were classified in biting midges,including 195 known species and 373 potentially new species,and 618 OPUs were classified in ticks,including 217 known species and 326 potentially new species.Surprisingly,OPUs with potentially pathogenicity were detected in both arthropod vectors,with 66 known species of biting midges reported to carry potential pathogens,including Asaia lannensis and Rickettsia bellii,compared to 50 in ticks,such as Acinetobacter lwoffii and Staphylococcus sciuri.We found that Proteobacteria was the most dominant group in both midges and ticks.Furthermore,the outcomes demonstrated that the microbiota of midges and ticks tend to be governed by a few highly abundant bacteria.Pantoea sp7 was predominant in biting midges,while Coxiella sp1 was enriched in ticks.Meanwhile,Coxiella spp.,which may be essential for the survival of Haemaphysalis longicornis Neumann,were detected in all tick samples.The identification of dominant species and pathogens of biting midges and ticks in this study serves to broaden our knowledge associated to microbes of arthropod vectors. Conclusion Biting midges and ticks carry large numbers of known and potentially novel bacteria,and carry a wide range of potentially pathogenic bacteria,which may pose a risk of infection to humans and animals.The microbial communities of midges and ticks tend to be dominated by a few highly abundant bacteria.
		                        		
		                        		
		                        		
		                        	
8.Statistical analysis of disability-adjusted life years for stomach and colorectal cancers in Changning District of Shanghai
Jing WU ; Lei ZHANG ; Yu JIANG ; Dandan TANG ; Yuxuan XIAO ; Yun ZHANG ; Honglan LI ; Wensui ZHAO ; Qinghua XIA ; Yongbing XIANG
Chinese Journal of Oncology 2024;46(2):168-176
		                        		
		                        			
		                        			Objectives:To analyze the status and temporal changes of disability-adjusted life year (DALY) for stomach and colorectal cancers among registered permanent residents in Changning District of Shanghai Municipality, and provide scientific basis for the prevention and treatment of stomach and colorectal cancers in this district.Methods:Using the cancer registration data of stomach and colorectal cancers from 2002 to 2019, we estimated the indices such as the DALYs, the DALY crude rates, the age-standardized DALY rates, etc. Then we used the Joinpoint regression model to calculate the average annual percent change (AAPC) and annual percent change (APC) to explore the temporal variations in different periods.Results:The DALYs of stomach and colorectal cancers in Changning District from 2002 to 2019 were 55 931 person years and 65 252 person years, respectively. The crude rates of DALY were 512.16/10 5 and 597.51/10 5, respectively. We observed a higher disease burden in men than in women, and the peak rate of DALY in stomach cancer was in the 75-79 years age group, while in colorectal cancer the rate was in the 85-years-or-older age group. Joinpoint regression analysis showed that from 2002 to 2019, the age-standardized DALY rate of stomach cancer showed a downward trend (AAPC=-3.86%, P<0.05), while the trend of colorectal cancer was not statistically significant(AAPC=-0.08%, P>0.05). However, the trends in the age-standardized DALY rates of colorectal cancer were different between males and females, with males showing an upward trend (AAPC=1.24%, P<0.05) and females showing a downward trend (AAPC=-1.67%, P<0.05). Conclusions:The DALY of stomach and colorectal cancers in Changning District of Shanghai showed a decreasing trend. Males and the middle-aged and elderly populations are still the key targets for disease prevention and control in this district.
		                        		
		                        		
		                        		
		                        	
9.Prediction of potential geographic distribution of Oncomelania hupensis in Yunnan Province using random forest and maximum entropy models
Zongya ZHANG ; Chunhong DU ; Yun ZHANG ; Hongqiong WANG ; Jing SONG ; Jihua ZHOU ; Lifang WANG ; Jiayu SUN ; Meifen SHEN ; Chunqiong CHEN ; Hua JIANG ; Jiaqi YAN ; Xiguang FENG ; Wenya WANG ; Peijun QIAN ; Jingbo XUE ; Shizhu LI ; Yi DONG
Chinese Journal of Schistosomiasis Control 2024;36(6):562-571
		                        		
		                        			
		                        			 Objective To predict the potential geographic distribution of Oncomelania hupensis in Yunnan Province using random forest (RF) and maximum entropy (MaxEnt) models, so as to provide insights into O. hupensis surveillance and control in Yunnan Province. Methods The O. hupensis snail survey data in Yunnan Province from 2015 to 2016 were collected and converted into O. hupensis snail distribution site data. Data of 22 environmental variables in Yunnan Province were collected, including twelve climate variables (annual potential evapotranspiration, annual mean ground surface temperature, annual precipitation, annual mean air pressure, annual mean relative humidity, annual sunshine duration, annual mean air temperature, annual mean wind speed, ≥ 0 ℃ annual accumulated temperature, ≥ 10 ℃ annual accumulated temperature, aridity and index of moisture), eight geographical variables (normalized difference vegetation index, landform type, land use type, altitude, soil type, soil textureclay content, soil texture-sand content and soil texture-silt content) and two population and economic variables (gross domestic product and population). Variables were screened with Pearson correlation test and variance inflation factor (VIF) test. The RF and MaxEnt models and the ensemble model were created using the biomod2 package of the software R 4.2.1, and the potential distribution of O. hupensis snails after 2016 was predicted in Yunnan Province. The predictive effects of models were evaluated through cross-validation and independent tests, and the area under the receiver operating characteristic curve (AUC), true skill statistics (TSS) and Kappa statistics were used for model evaluation. In addition, the importance of environmental variables was analyzed, the contribution of environmental variables output by the models with AUC values of > 0.950 and TSS values of > 0.850 were selected for normalization processing, and the importance percentage of environmental variables was obtained to analyze the importance of environmental variables. Results Data of 148 O. hupensis snail distribution sites and 15 environmental variables were included in training sets of RF and MaxEnt models, and both RF and MaxEnt models had high predictive performance, with both mean AUC values of > 0.900 and all mean TSS values and Kappa values of > 0.800, and significant differences in the AUC (t = 19.862, P < 0.05), TSS (t = 10.140, P < 0.05) and Kappa values (t = 10.237, P < 0.05) between two models. The AUC, TSS and Kappa values of the ensemble model were 0.996, 0.954 and 0.920, respectively. Independent data verification showed that the AUC, TSS and Kappa values of the RF model and the ensemble model were all 1, which still showed high performance in unknown data modeling, and the MaxEnt model showed poor performance, with TSS and Kappa values of 0 for 24%(24/100) of the modeling results. The modeling results of 79 RF models, 38 MaxEnt models and their ensemble models with AUC values of > 0.950 and TSS values of > 0.850 were included in the evaluation of importance of environmental variables. The importance of annual sunshine duration (SSD) was 32.989%, 37.847% and 46.315% in the RF model, the MaxEnt model and their ensemble model, while the importance of annual mean relative humidity (RHU) was 30.947%, 15.921% and 28.121%, respectively. Important environment variables were concentrated in modeling results of the RF model, dispersed in modeling results of the MaxEnt model, and most concentrated in modeling results of the ensemble model. The potential distribution of O. hupensis snails after 2016 was predicted to be relatively concentrated in Yunnan Province by the RF model and relatively large by the MaxEnt model, and the distribution of O. hupensis snails predicted by the ensemble model was mostly the joint distribution of O. hupensis snails predicted by RF and MaxEnt models. Conclusions Both RF and MaxEnt models are effective to predict the potential distribution of O. hupensis snails in Yunnan Province, which facilitates targeted O. hupensis snail control. 
		                        		
		                        		
		                        		
		                        	
10.Statistical analysis of disability-adjusted life years for stomach and colorectal cancers in Changning District of Shanghai
Jing WU ; Lei ZHANG ; Yu JIANG ; Dandan TANG ; Yuxuan XIAO ; Yun ZHANG ; Honglan LI ; Wensui ZHAO ; Qinghua XIA ; Yongbing XIANG
Chinese Journal of Oncology 2024;46(2):168-176
		                        		
		                        			
		                        			Objectives:To analyze the status and temporal changes of disability-adjusted life year (DALY) for stomach and colorectal cancers among registered permanent residents in Changning District of Shanghai Municipality, and provide scientific basis for the prevention and treatment of stomach and colorectal cancers in this district.Methods:Using the cancer registration data of stomach and colorectal cancers from 2002 to 2019, we estimated the indices such as the DALYs, the DALY crude rates, the age-standardized DALY rates, etc. Then we used the Joinpoint regression model to calculate the average annual percent change (AAPC) and annual percent change (APC) to explore the temporal variations in different periods.Results:The DALYs of stomach and colorectal cancers in Changning District from 2002 to 2019 were 55 931 person years and 65 252 person years, respectively. The crude rates of DALY were 512.16/10 5 and 597.51/10 5, respectively. We observed a higher disease burden in men than in women, and the peak rate of DALY in stomach cancer was in the 75-79 years age group, while in colorectal cancer the rate was in the 85-years-or-older age group. Joinpoint regression analysis showed that from 2002 to 2019, the age-standardized DALY rate of stomach cancer showed a downward trend (AAPC=-3.86%, P<0.05), while the trend of colorectal cancer was not statistically significant(AAPC=-0.08%, P>0.05). However, the trends in the age-standardized DALY rates of colorectal cancer were different between males and females, with males showing an upward trend (AAPC=1.24%, P<0.05) and females showing a downward trend (AAPC=-1.67%, P<0.05). Conclusions:The DALY of stomach and colorectal cancers in Changning District of Shanghai showed a decreasing trend. Males and the middle-aged and elderly populations are still the key targets for disease prevention and control in this district.
		                        		
		                        		
		                        		
		                        	
            
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