This case report presents a 16-year-old male patient with deficiency of adenosine deaminase 2(DADA2). The patient had a history of Raynaud′s phenomenon with digital ulcers since childhood. As the disease progressed, the patient developed retinal vasculitis, intracranial hemorrhage, skin necrosis, severe malnutrition, refractory hypertension, and gastrointestinal bleeding. Genetic testing revealed compound heterozygous mutations in the ADA2 gene (paternal c.1072G > A pathogenic mutation + maternal c.1065C > A variant of unknown clinical significance). ADA2 enzyme activity was significantly reduced (0.1 U/L). A multidisciplinary treatment team developed an individualized plan to address key issues, including nutritional support, blood pressure control, rehabilitation, pain management, and balancing anticoagulation/bleeding risks. After systematic intervention, the patient′s condition showed partial improvement. This case reveals clinical challenges such as anticoagulation decisions and nutritional support of DADA2. It also emphasizes the importance of early molecular diagnosis, tumor necrosis factor-α inhibitor targeted therapy, and multidisciplinary collaboration in management, underlining the core value of precision medicine in rare disease management.

Objective:To explore effect of information management mode of full lifecycle for medical equipment on the basis of Service-Oriented Architecture(SOA)on the information management for information management of full lifecycle in hospital.Methods:An information management mode of full lifecycle for medical equipment was adopted to conduct information management for full lifecycle of medical equipment,so as to solve a series of problems included perception for the situation of equipment,and early warning for fault,and so on.A total of 100 medical equipment in clinical use at The First Rongjun Youfu Hospital of Shandong Province from February 2023 to February 2024 were selected for the study.In them,50 equipment were managed under a continuous improvement mode,while the other 50 equipment were managed by using a SOA-based information management mode of full lifecycle(information management mode).The score of management quality for equipment,management standardization and operational quality of the two kinds of management modes were compared.A self-designed satisfaction questionnaire of using and managing equipment was used to survey the satisfactions of medical engineers,operators of equipment,and staffs of equipment department in using and managing equipment,who involved in usage and management for equipment.Results:Under the information management mode,the scores for usage of equipment,the return efficiency,safety performance,maintenance quality,cleaning quality,and overall evaluation were respectively(22.98±2.11),(23.69±1.65),(22.74±1.36),(23.65±1.54),and(93.01±4.65),all of which were significantly higher than those of the continuous improvement mode,with statistically significant differences(t=6.672,11.428,9.256,12.976,13.182,P<0.05).The standardizations of usage operation of equipment,cleaning and disinfection,safety monitoring,and quality control of adopting information management mode were higher than those of adopting continuous improvement mode(x2=13.709,11.679,11.284,15.120,P<0.05).The failure rates of surgical equipment,therapeutic equipment,diagnostic equipment,dialysis equipment,and emergency equipment of adopting information management mode were all lower than those of adopting continuous improvement mode,with statistically significant differences(x2=16.872,13.878,17.156,11.679,12.805,P<0.05).Satisfaction scores of medical engineers,operators for equipment,and staffs of equipment department of adopting information management mode were significantly higher than those of adopting the continuous improvement mode,with statistically significant differences(t=16.872,13.878,17.156,P<0.05).Conclusion:The application of a SOA-based information management mode of full lifecycle for medical equipment in management for medical equipment can enhance management level for equipment,and improve operational quality of equipment,and enhance safety and utilization efficient of using equipment.

Objective:To explore effect of information management mode of full lifecycle for medical equipment on the basis of Service-Oriented Architecture(SOA)on the information management for information management of full lifecycle in hospital.Methods:An information management mode of full lifecycle for medical equipment was adopted to conduct information management for full lifecycle of medical equipment,so as to solve a series of problems included perception for the situation of equipment,and early warning for fault,and so on.A total of 100 medical equipment in clinical use at The First Rongjun Youfu Hospital of Shandong Province from February 2023 to February 2024 were selected for the study.In them,50 equipment were managed under a continuous improvement mode,while the other 50 equipment were managed by using a SOA-based information management mode of full lifecycle(information management mode).The score of management quality for equipment,management standardization and operational quality of the two kinds of management modes were compared.A self-designed satisfaction questionnaire of using and managing equipment was used to survey the satisfactions of medical engineers,operators of equipment,and staffs of equipment department in using and managing equipment,who involved in usage and management for equipment.Results:Under the information management mode,the scores for usage of equipment,the return efficiency,safety performance,maintenance quality,cleaning quality,and overall evaluation were respectively(22.98±2.11),(23.69±1.65),(22.74±1.36),(23.65±1.54),and(93.01±4.65),all of which were significantly higher than those of the continuous improvement mode,with statistically significant differences(t=6.672,11.428,9.256,12.976,13.182,P<0.05).The standardizations of usage operation of equipment,cleaning and disinfection,safety monitoring,and quality control of adopting information management mode were higher than those of adopting continuous improvement mode(x2=13.709,11.679,11.284,15.120,P<0.05).The failure rates of surgical equipment,therapeutic equipment,diagnostic equipment,dialysis equipment,and emergency equipment of adopting information management mode were all lower than those of adopting continuous improvement mode,with statistically significant differences(x2=16.872,13.878,17.156,11.679,12.805,P<0.05).Satisfaction scores of medical engineers,operators for equipment,and staffs of equipment department of adopting information management mode were significantly higher than those of adopting the continuous improvement mode,with statistically significant differences(t=16.872,13.878,17.156,P<0.05).Conclusion:The application of a SOA-based information management mode of full lifecycle for medical equipment in management for medical equipment can enhance management level for equipment,and improve operational quality of equipment,and enhance safety and utilization efficient of using equipment.

Objective To investigate the recurrence of immune thrombocytopenia(ITP)in children and to establish a predictive model.Methods A total of 288 children with ITP admitted to Children's Hospital of Wujiang District and Children's Hospital Affiliated to Suzhou University from January 2018 to April 2022 were collected.The factors potentially related to the recurrence of ITP in children were screened.The children in the model group were divided into 2 groups according to the presence or absence of recurrence and the indicators of the 2 groups were compared.After screening the potential influencing factors by LASSO regression and the independent influencing factors of relapse in children with ITP patients by Logstic regression analysis,we constructed a column-line graph model by using R language and validated it.Results A total of 37(18.47%)of 201 patients in the model group experienced relapse.The age,blood type,duration of disease before treatment,antecedent infections,bleeding,initial treatment regimen,antinuclear antibody titer,initial count and mean platelet volume,initial platelet distri-bution width,initial peripheral blood lymphocyte count and time length to effective platelet count after treatment were found in the recurrence group versus the non-recurrence group The difference was statistically significant(P＜0.05).The results of multifactorial logistic regression analysis performed on the basis of LASSO regression showed that blood type,duration of illness before treatment,antecedent infection,initial treatment regimen,ini-tial peripheral blood lymphocyte count,and time to effective platelet count after treatment were independent influ-ences on the conversion of cough variant asthma to classic asthma in children.Based on the results of the multifac-torial analysis,a column chart model for predicting ITP recurrence in children was developed in R.The results of the receiver operating characteristic(ROC)analysis showed that the area under curve(AUC)of the column chart model for predicting ITP recurrence in children in the modeling group was 0.867[95%CI(0.796,0.938)]with a sensitivity of 84.2%and a specificity of 73.1%,and that in the validation group,the AUC was 0.838[95%CI(0.765),0.911]with a sensitivity of 82.3%and a specificity of 78.4%,0.911)]sensitivity was 82.3%and specificity was 78.4%.The Bootstrap method was used to repeat the sampling 1000 times,and the validation group was used for validation.The results of the calibration curve showed that the prediction curves of the model group and the validation group were basically fitted with the standard curve,suggesting that the model prediction accuracy was high.The results of the decision curve analysis of the model group showed that the net benefit rate of patients was greater than zero when the probability threshold of the column line graph model of pre-dicting ITP recurrence in children was 0.15-0.75.Conclusions ITP recurrence in children is mainly affected by the patient's age,blood type,and pre-treatment course of the disease,and the column-line diagram model based on these factors has a high accuracy and differentiation for ITP recurrence in parenting children.

Objective To explore the diagnostic value of lymphocyte subpopulations combined with chemokines in children with immunologic thrombocytopenic purpura(ITP).Methods 132 children with proposed diagnosis of ITP were collected,and the children were divided into ITP and non-ITP groups according to the diagnostic results of ITP-related clinical diagnostic criteria.6 ml of peripheral venous blood was drawn,the levels of CD4+CD8+and CD3+were detected using flow cytometry,and the levels of chemokine(C-C motif)ligand 5(CCL5),Recombi-nant Chemokine(C-X-C Motif)Ligand 1(CXCL11),and monocyte chemotactic protein-1(MCP-1)were detec-ted using enzyme-linked immunosorbent assay,the blood platelet(PLT)was measured by a fully automated cell an-alyzer.The children were divided into ITP and non-ITP groups according to the clinical diagnostic criteria related to ITP.The lymphocyte subpopulations and chemokine levels of the two groups of children were compared,and the correlation between lymphocyte subpopulations and chemokine levels and PLT was analyzed.The ROC method was used to evaluate the diagnostic efficacy of individual and combined detection of each indicator for ITP.Results The levels of CD4+and CD3+in the ITP group were lower than those in the non ITP group(P<0.05),while the levels of CD8+were higher than those in the non ITP group(P<0.05).The levels of CCL5,CXCL11,and MCP-1 in the ITP group were higher than those in the non ITP group(P<0.05).The correlation analysis results showed that CD4+,CD3+and platelet count were positively correlated in the ITP group(P<0.05),while CD8+,CCL5,CXCL11,MCP-1 were negatively correlated with PLT(P<0.05).The ROC analysis results showed that the cut-off values of CD4+,CD8+,CD3+,CCL5,CXCL11,and MCP-1 for the diagnosis of ITP in children were 27.13%,24.02%,59.88%,41.02 ng/L,30.18 ng/L,and 188.27 ng/L,respectively.The AUC values were 0.893,0.880,0.629,0.801,0.892,and 0.751,respectively,The AUC of the parallel diagnosis(meaning that one or more of CD4+,CD3+was below the cut-off value and/or one or more of CD8+,CCL5,CXCL11,MCP-1 was above the cut-off value at the time of parallel testing)was 0.967,indicating that one or more of them was lower than the cut off value and/or one or more of them was higher than the cut off value when tested separately.Its diag-nostic efficacy was higher than that of each indicator tested separately(P<0.05).Conclusion There are signifi-cant differences in lymphocyte subpopulations and chemokines between pediatric ITP patients and non-ITP patients.CD4+,CD8+,CD3+,CCL5,CXCL11,and MCP-1 can be used for the diagnosis of pediatric ITP.Combined de-tection of various indicators can improve detection efficiency.

Objective:To explore the application of scenario simulation teaching based on PBL in communication skills training of hematology students in Children's Hospital.Methods:The training of doctor-patient communication skills was conducted among trainees who had the standardized residency training at the Department of Hematology of the Children's Hospital of Soochow University. All the residents were randomized into the control group and observation group by lottery, with 24 residents in each group. The control group adopted the traditional narrative teaching method, and the observation group adopted PBL combined with scenario simulation teaching method. The Liverpool communication skills assessment scale (LCSAS) was used to compare the differences between the two groups before and after training, and the differences between the two groups after training. Then the degree of residents' recognition of these two teaching methods was investigated. Finally, the examination results were used to evaluate knowledge mastery of doctors in department of hematology. SPSS 20.0 was used for Chi-square test and t-test. Results:LCSAS scores of the two groups before training were respectively (11.61±2.21) and (11.95±2.22), with no statistically significant difference ( P >0.05). After PBL-based scenario simulation teaching and training in the observation group, the LCSAS score of the observation group (27.41±2.53) was higher than that of the control group (23.30±1.81), and the difference between the two groups was statistically significant ( P<0.05). Questionnaire survey results showed that the favorable rating rate of PBL-based scenario simulation teaching was 91.67% (22/24), higher than that of the traditional narrative teaching method [62.50% (15/24)], and the difference was statistically significant ( P<0.05). The examination of students' mastery of professional knowledge showed that after the PBL-based scenario simulation teaching and training, the trainees had a better grasp of knowledge and a higher score, with excellence rate of 91.67% (22/24）, which was higher than 66.67% (16/24) of the control group, with a statistically significant difference ( P<0.05). Conclusion:The scenario simulation teaching based on PBL could improve the communication ability and professional knowledge of trainees taking standardized residency training in the department of hematology, and the trainees are highly satisfied with this teaching method.

Objective:To construct a surface display system containing various lengths of the Ag43 passenger domain for an optimal bacterial surface display of foreign protein HPV16L1.Methods:(1) Ag43 gene sequences of different lengths were inserted into pET22b vector to construct four Ag43 surface display vectors (Ag43/138, Ag43/551, Ag43/552 and Ag43/700) using PCR and subcloning strategy. (2) The generation of four HPV16L1-Ag43 fusion constructs was completed by PCR and subcloning methods. (3) HPV16L1-Ag43 fusion proteins were expressed and analyzed by SDS-PAGE. (4) The surface exposure of HPV-16L1 was verified using trypsin digestion.Results:PCR analysis and sequencing results showed that Ag43 surface display vectors and HPV16L1-Ag43 fusions were constructed successfully. SDS-PAGE showed that the expression of HPV16L1-Ag43 fusion proteins could be induced with 0.2 mmol/L IPTG and the protein content was reduced after the cells were treated with trypsin, especially the content of Ag43/700-HPV16L1 that showed a drastic reduction.Conclusions:The Ag43 surface display system was successfully constructed and could be used for a successful display of HPV16L1. This study also showed that Ag43/700 comprising only the α-helix and the β-barrel of Ag43 provided an optimal surface display for HPV16L1.

Objective:To evaluate the efficacy and safety of sequential platinum regimen in young patients with diffuse large B-cell lym-phoma (DLBCL). Methods:Newly diagnosed young patients with DLBCL, who were hospitalized from January 2005 to June 2012 in the Affiliated Cancer Hospital of Zhengzhou University, were selected according to the requirements. The patients were divided into stan-dard and sequential platinum regimen groups. The remission rates were compared usingχ2 test, whereas the five-year survival rates between the two groups were compared using the Kaplan–Meier method. Multivariate survival analysis was performed using the Cox proportional regression. Subgroup analysis was conducted to select candidate patients for the sequential platinum regimen. Results:A total of 331 patients were enrolled in the study, in which 129 were provided with sequential platinum regimen and 202 were provided with the standard regimen. Sequential regimen yielded higher rates of complete remission (80%vs. 63%, P=0.001), five-year progres-sion-free survival (PFS;60%vs. 50%, P=0.014), and overall survival (OS;70%vs. 58%, P=0.016) than the standard regimen. Multivariate analysis revealed that sequential regimen was an independent prognostic factor for PFS (hazard ratio HR=0.635, P=0.012) and OS (HR=0.625, P=0.021). Subgroup analysis showed that patients with good prognosis and patients who did not receive rituximab benefited more from the sequential platinum regimen. Sequential platinum regimen did not increase the occurrence of adverse effects com-pared with the standard regimen. Conclusion:Sequential platinum regimen is a safe treatment that can improve the survival of young patients with DLBCL. Patients with good prognosis and patients who did not receive rituximab can benefit more from the treatment with sequential platinum regimen.